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Mutations in the nuclear-encoded mitochondrial maintenance gene RRM2B are an important cause of familial mitochondrial disease in both adults and children and represent the third most common cause of multiple mitochondrial DNA deletions in adults, following POLG [polymerase (DNA directed), gamma] and PEO1 (now called C10ORF2, encoding the Twinkle helicase) mutations. However, the clinico-pathological and molecular features of adults with RRM2B-related disease have not been clearly defined. In this multicentre study of 26 adult patients from 22 independent families, including five additional cases published in the literature, we show that extra-ocular neurological complications are common in adults with genetically confirmed RRM2B mutations. We also demonstrate a clear correlation between the clinical phenotype and the underlying genetic defect. Myopathy was a prominent manifestation, followed by bulbar dysfunction and fatigue. Sensorineural hearing loss and gastrointestinal disturbance were also important findings. Severe multisystem neurological disease was associated with recessively inherited compound heterozygous mutations with a mean age of disease onset at 7 years. Dominantly inherited heterozygous mutations were associated with a milder predominantly myopathic phenotype with a later mean age of disease onset at 46 years. Skeletal muscle biopsies revealed subsarcolemmal accumulation of mitochondria and/or cytochrome c oxidase-deficient fibres. Multiple mitochondrial DNA deletions were universally present in patients who underwent a muscle biopsy. We identified 18 different heterozygous RRM2B mutations within our cohort of patients, including five novel mutations that have not previously been reported. Despite marked clinical overlap between the mitochondrial maintenance genes, key clinical features such as bulbar dysfunction, hearing loss and gastrointestinal disturbance should help prioritize genetic testing towards RRM2B analysis, and sequencing of the gene may preclude performance of a muscle biopsy.
Approximately one-half of all adult mitochondrial diseases result from genetic dysfunction of the nuclear-encoded mitochondrial genes. A significant number of these cases are due to disruption of genes involved in maintenance (replication and repair) of mitochondrial DNA, leading to qualitative (accumulation of multiple mitochondrial DNA deletions) and/or quantitative (depletion of mitochondrial DNA copy number) downstream mitochondrial genomic effects. To date, almost all known nuclear maintenance genes fall into one of two categories: (i) genes whose protein products function directly at the mitochondrial DNA replication fork (Van Goethem et al., 2001; Spelbrink et al., 2001; Longley et al., 2006); or (ii) genes that encode proteins involved in supplying mitochondria with deoxyribonucleoside triphosphate (dNTP) pools required for DNA replication (Nishino et al., 1999; Kaukonen et al., 2000; Mandel et al., 2001; Saada et al., 2001; Bourdon et al., 2007; Ostergaard et al., 2007; Fratter et al., 2011). The most common presenting neurological feature seen in adults with mitochondrial DNA maintenance disorders is progressive external ophthalmoplegia (PEO) and ptosis. Of the 12 known maintenance genes, eight have been associated with PEO, although the clinical phenotype is not necessarily restricted to the extra-ocular muscles.
Ribonucleotide reductase M2B (TP53 inducible), RRM2B (MIM 604 712), encodes the p53-inducible small subunit (p53R2) of ribonucleotide reductase, a heterotetrameric enzyme that catalyses de novo syntheses of dNTPs by direct reduction of ribonucleoside diphosphates to their corresponding deoxyribonucleoside diphosphates. This process supplements the dNTPs produced by the mitochondrion's own dNTP salvage pathway, defects of which cause many of the mitochondrial DNA depletion syndromes (Rahman and Poulton, 2009), and is essential for mitochondrial DNA synthesis. Transcription of RRM2B is tightly regulated by the tumour suppressor protein p53. Mutations in the gene have been associated with both recessively and dominantly inherited mitochondrial disease. Spliced variant transcripts have also been reported (Spinazzola et al., 2009).
The first reported human diseases linked to RRM2B dysfunction were associated with mitochondrial DNA depletion (Bourdon et al., 2007; Bornstein et al., 2008; Acham-Roschitz et al., 2009; Kolberg et al., 2009; Spinazzola et al., 2009) and caused severe, early-onset, multisystem disease with infant mortality. RRM2B mutations have since been recognized to cause adult-onset syndromes. This was first demonstrated in a patient with mitochondrial neurogastrointestinal encephalopathy and mitochondrial DNA depletion (Shaibani et al., 2009). Tyynismaa et al. (2009) later published about a large autosomal-dominant PEO family with multiple mitochondrial DNA deletions whose disease was not explained by mutations in any of the known autosomal-dominant PEO genes. The disease locus in the family was mapped using genome-wide linkage and found to contain RRM2B, and a heterozygous nonsense mutation in exon 9 was detected and demonstrated in another unrelated family. The mutant messenger RNA was shown to escape nonsense-mediated decay and resulted in a truncated protein that was postulated to cause a dominant-negative or gain-of-function effect on the heterotetrameric structure of the ribonucleotide reductase enzyme. The mechanistic importance of truncating exon 9 mutations was further expanded by Fratter et al. (2011), who showed that three further novel pathogenic variants in exon 9 caused familial autosomal-dominant PEO in seven unrelated probands. In addition, clinically more severe, recessively inherited, compound heterozygous mutations were demonstrated. Kearns–Sayre syndrome resulting from compound heterozygous RRM2B mutations with secondary multiple mitochondrial DNA deletions has also been reported, thus confirming the more severe nature of recessively inherited adult RRM2B mutations and expanding the clinical phenotype associated with RRM2B-related mitochondrial disease beyond that of PEO and PEO-plus syndromes (Pitceathly et al., 2011).
Despite emerging evidence that RRM2B mutations are the third most common cause of multiple mitochondrial DNA deletions in adults, following POLG and PEO1 (now known as C10ORF2) (Fratter et al., 2011; Pitceathly et al., 2011), and RRM2B dysfunction being an important cause of neurological disease, the adult phenotypic and molecular spectrum is not fully understood. We, therefore, conducted a systematic clinical and molecular study of adult patients with RRM2B mutation(s) to define the phenotypic spectrum of adult RRM2B-related mitochondrial disease and establish any genotype–phenotype correlations.
We selected a cohort of adult patients who had been referred to the NHS Specialized Services-funded Mitochondrial Diagnostic Centres in Newcastle and Oxford, UK, or the Mitochondrial Centre in Halle, Germany, for investigation of suspected mitochondrial disease, owing to their clinical presentation, evidence of a mitochondrial biochemical defect and/or presence of mitochondrial DNA deletions. RRM2B sequencing was performed when initial screens for POLG, POLG2, PEO1 and SLC25A4 were found to be negative.
The subject's consent was obtained according to the Declaration of Helsinki, and institutional ethical committee approval was obtained at each centre in which the work was performed.
We conducted a systematic review of the literature to identify all previously published adult cases with confirmed RRM2B mutations. These were included in our analysis to determine the complete clinical spectrum and genotype–phenotype correlates seen in adult RRM2B-related mitochondrial disease (Shaibani et al., 2009; Tyynismaa et al., 2009; Fratter et al., 2011; Pitceathly et al., 2011; Takata et al., 2011).
To define the extent and severity of patient's mitochondrial disease, a neurologist at each centre comprehensively assessed each patient harbouring pathogenic RRM2B mutations and/or examined their medical records.
Skeletal muscle biopsies were collected from 21 patients with pathogenic RRM2B mutations. Cryostat sections (10 µm) were cut from transversely oriented muscle blocks and subjected to cytochrome c oxidase (COX), succinate dehydrogenase (SDH) and sequential COX-SDH histochemical staining to assess the numbers of COX-deficient fibres as a measure of respiratory chain deficiency, as previously described (Taylor et al., 2004). The SDH reaction was used to ascertain the number of fibres exhibiting increased levels of enzyme activity in the subsarcolemmal region, namely, 'ragged-blue' fibres, in addition to data obtained from the histological modified Gomori trichrome stain.
Where available, total muscle DNA was extracted by standard techniques and screened for mitochondrial DNA rearrangements by Southern blotting and/or long-range PCR protocols, as described previously (Murphy et al., 2008). The presence of clonally expanded mitochondrial DNA deletions in individual COX-positive and COX-deficient fibres was further screened in muscle biopsies from nine patients with either dominantly inherited or recessively inherited RRM2B mutations (Patients 1.1, 3, 5, 9, 10, 14, 19 and 20), by previously published protocols, using primers and TaqMan® probes to assess the simultaneous amplification of the MTND1 (rarely deleted) and MT-ND4 (often deleted) genes (He et al., 2002; Krishnan et al., 2007). Absence of mitochondrial DNA depletion in muscle was confirmed in all cases by real-time quantitative PCR, as described previously (Blakeley et al., 2008).
The coding exons and intron–exon boundaries of RRM2B (GenBank accession number NM_015713) were sequenced, as previously described (Tyynismaa et al., 2009). RRM2B exon copy number (exons 1–8) was assessed by multiplex ligation-dependent probe amplification (MRC-Holland kit P089-A1) in all patients with dominantly inherited heterozygous missense RRM2B mutations. Total RNA from patients with the c.48G>A RRM2B variant (Patients 1, 1.1 and 2) was extracted from Ethylenediaminetetraacetic acid-blood with the QIAamp® RNA Blood Mini kit (Qiagen) and reverse transcribed using the ThermoScript™ RT-PCR system (Invitrogen). The resulting complementary DNA was PCR-amplified across the RRM2B exon 1–2 boundary using exonic primers, leading to a normally spliced 303-bp product. The PCR products were separated by agarose gel electrophoresis under standard conditions. Gel stabs of discreet bands were taken for secondary PCR using the same primers, which were M13-tagged for subsequent dideoxy Sanger sequencing using universal M13 primers, BigDye® Terminator kit 3.1 (Applied Biosystems) and capillary electrophoresis on an ABI Prism 3730 Genetic Analyser.
The 2.6 Å X-ray crystal structure of human p53R2 (Research Collaboratory for Structural Bioinformatics Protein Data Bank: reference 3HF1) (Smith et al., 2009) was viewed in ViewerLite v.4.2 (Accelrys Inc.), and the amino acids known to have been substituted were isolated and saved as a separate pdb file. The p53R2 crystal structure and the isolated amino acids were then rendered using PyMOL v.0.97 (Warren L. DeLano 'The PyMOL Molecular Graphics System' DeLano Scientific LLC, http://www.pymol.org).
Clinical data are presented on 31 patients (Table 1). Twenty-six patients were identified by the NHS Specialized Services-funded Mitochondrial Diagnostic Centres in Newcastle and Oxford, UK, or the Mitochondrial Centre in Halle, Germany (Newcastle–Oxford–Halle cohort), and are described here in greater clinical and molecular detail. Clinical information from five further patients was obtained from the literature review. One previously published case (Patient 12, Fratter et al., 2011) was omitted from the current analysis, as the pathogenic nature of the reported heterozygous mutation was uncertain after segregation studies.
The clinical and molecular features of all 26 patients from our Newcastle–Oxford–Halle cohort are summarized in Table 2. Molecular data from 11 of these subjects have been previously reported in brief (Fratter et al., 2011). The mean whole-group age at onset was 40 years (age range birth–70 years); patients with autosomal-recessive PEO presented considerably earlier at 7 years (age range birth–14 years), whereas those with autosomal-dominant PEO presented later in the fifth decade of life (mean age of onset 46 years; age range 15–70 years).
A systematic review of the literature to date (June 1, 2012) revealed published data on 18 RRM2B-positive adult families (Supplementary Table 1). Thirty-three individuals have been extensively genotyped and harbour RRM2B gene mutations characterized by either autosomal-recessive mitochondrial DNA depletion syndrome (Shaibani et al., 2009) or recessive and dominant mutations that cause the accumulation of multiple mitochondrial DNA deletions (Tyynismaa et al., 2009; Fratter et al., 2011; Pitceathly et al., 2011; Takata et al., 2011).
Evaluation of the clinical features of all 31 adult patients harbouring pathogenic RRM2B mutations showed that PEO was universal and frequently associated with ptosis (28 patients). Neuromuscular features were common and included proximal muscle weakness (16 patients), bulbar dysfunction (13 patients) and fatigue (11 patients). Additional neurological manifestations included ataxia (12 patients) and sensorineural hearing loss (11 patients). Non-neurological sequelae such as gastrointestinal disturbance, including irritable bowel syndrome-like symptoms and low body mass index (six patients), and endocrinopathy, specifically hypothyroidism, hypoparathyroidism, diabetes and hypogonadism (seven patients), were also important clinical findings. Malignancy was detected in two patients (Patients 12 and 18).
Other less common problems included cognitive impairment (four patients); cardiac dysfunction, including non-fatal arrhythmia and diastolic dysfunction (two patients); encephalopathy and stroke-like events (three patients); cataracts (two patients); migraine (two patients); renal disturbance (three patients); distal muscle weakness (one patient); neuropathy (three patients); pigmentary retinopathy (two patients); short stature (one patient); glaucoma (one patient) and depression (three patients).
Twenty-one patients with confirmed RRM2B mutations underwent a diagnostic muscle biopsy for the investigation of suspected mitochondrial disease. Patient 6.1 was diagnosed on the basis of RRM2B gene screening of a blood-derived DNA sample without performing a muscle biopsy. All showed histochemical evidence of a COX mosaic defect, with many exhibiting evidence of subsarcolemmal mitochondrial accumulation (ragged-red or ragged-blue fibres) after Gomori trichrome staining or SDH enzyme histochemistry. The severity of the associated COX mosaic defect varied widely, with a more pronounced histochemical defect associated with autosomal-recessive RRM2B mutations than autosomal-dominant mutations (Fig. 1).
All 21 patients who underwent muscle biopsy had multiple mitochondrial DNA deletions detectable either by long-range PCR assays across the major mitochondrial DNA arc (Fig. 2) or by Southern blot analysis (undertaken in 11 of the 21 patients, with positive findings reported in all). A small number of patients (five patients with autosomal-dominant missense or truncating RRM2B mutations, one patient with an autosomal-dominant splicing defect and two patients with autosomal-recessive RRM2B mutations) were also investigated by real-time PCR to further characterize the presence of clonally expanded mitochondrial DNA deletions within single muscle fibres (Fig. 2). In all cases, the majority of COX-deficient fibres revealed very high levels (>80% mutated mitochondrial DNA) of clonally expanded mitochondrial DNA deletion involving the MT-ND4 gene region (He et al., 2002), whereas all COX-positive reacting fibres had lower levels of mitochondrial DNA deletion. No difference in the distribution of mitochondrial DNA deletion levels was observed between different RRM2B mutation types.
We identified 18 (12 dominantly and six recessively inherited) different RRM2B mutations within our cohort of patients, including five novel mutations that have not previously been reported (Fig. 3). Thirteen mutations were missense variants predicted to alter conserved amino acids (Supplementary Fig. 1), four were truncating mutations in exon 9 and one was a novel c.48G>A variant predicted to cause aberrant splicing.
Eleven patients (from nine families) were found to have truncating mutations in exon 9 of RRM2B, three with the p.Leu317X mutation, one with a p.Glu318X mutation, five with a p.Asn322LysfsX4 frameshift mutation and two patients from one family (Patient 17 and her clinically affected daughter, Patient 17.1) with the p.Arg327X mutation, which was the first mutation in this gene to be described as a cause of autosomal-dominant PEO (Tyynismaa et al., 2009). All nine probands had affected family members and a family history consistent with a dominantly inherited disorder (Table 2). Eight patients (from seven families) harboured single heterozygous RRM2B missense changes, implying autosomal-dominant inheritance. In all seven families, multiplex ligation-dependent probe amplification was used to exclude exonic copy number variation in trans, with further supporting evidence of pathogenicity provided by the fact that these changes affected conserved residues (Supplementary Fig. 1). Four of these RRM2B mutations were unreported. Patient 4 harboured a novel p.Arg41Trp mutation, and although there was no relevant family history to support dominant transmission, mutation of this particular amino acid (p.Arg41Gln) was reported in another patient (Patient 3). Patient 5 had a novel p.Asp70Asn mutation and a clinically affected sister. Patient 18 had a novel mutation (p.Ala349Gly) in exon 9, which is a recognized mutation hotspot (Fratter et al., 2011). The single heterozygous p.Ile224Ser mutation reported in Patient 8 is of particular interest, given that it has previously been reported as a recessive RRM2B mutation in a case of mitochondrial DNA depletion myopathy (Bornstein et al., 2008) and, as such, seems to be able to behave as either a recessive or dominant allele. Similarly, p.Arg41Gln and p.Arg211Lys function as dominant alleles in Patients 3 and 7, respectively, but have also been identified in compound heterozygotes with more severe recessively inherited disease (Pitceathly et al., 2011; and Patient 21). Four patients harboured two recessively inherited compound heterozygous RRM2B mutations associated with an earlier onset of disease and more pronounced COX histochemical defects (Fig. 1 and Table 2). The p.Thr144Ile and p.Gly273Ser mutations were reported in trans in two of the four patients, whereas the p.Arg186Gly and p.Thr218Ile mutations (Patient 20) were both novel. Within this family, the p.Thr218Ile mutation was detected in the heterozygous state in the clinically unaffected mother and a sister, whereas the second heterozygous mutation, p.Arg186Gly, was identified in another clinically unaffected sister. Segregation studies were also performed in the family of Patient 19; the p.Gly273Ser mutation was seen in the heterozygous state in the clinically unaffected mother, whereas a clinically unaffected sister was heterozygous for the p.Thr144Ile mutation alone. Paternal testing was not performed.
Finally, an unreported c.48G>A RRM2B variant was identified in three patients (Patients 1, 1.1 and 2). This is not predicted to alter the amino acid sequence, resulting in a predicted p.Glu16Glu synonymous change. However, it was noted that the c.48G>A variant occurs at the last nucleotide of exon 1 and was therefore considered likely to result in aberrant splicing (Fig. 4). Analysis of blood RNA extracted from Patients 1, 1.1 and 2 across the exon 1–2 junction demonstrated that normally spliced RNA arises exclusively from the normal c.48G allele (Fig. 4). Furthermore, the three patients had additional RNA species associated with the c.48G>A allele, in which various portions of the 5′ region of intron 1 were retained, resulting in the introduction of a termination codon, 26 codons beyond exon 1. All three patients had RNA incorporating the first 268 nucleotides of intron 1. Patient 1.1 also had RNA with the first 518 nucleotides of intron 1, and Patient 1 also had RNA with the first 467 and 518 nucleotides of intron 1 (Fig. 4). Thus, these results demonstrate that c.48G>A abolishes normal splicing of exons 1 to 2, leading to partial intron retention and premature termination of translation, thereby confirming pathogenicity of c.48G>A.
RRM2B mutations are emerging as one of the leading causes of both paediatric and adult-onset mitochondrial disease associated with disruption of mitochondrial DNA maintenance. RRM2B mutations represent the third most common cause of Mendelian PEO and multiple mitochondrial DNA deletions in adults (13%), following mutations in POLG (27%) and PEO1 (14%), based on data from both Oxford and Newcastle centres. It is clear there is significant clinical overlap between the multiple mitochondrial maintenance genes. In our cohort, ophthalmoparesis was universal, usually severe and often associated with ptosis (90% of patients). Other prominent myopathic features included proximal muscle weakness (52% of patients) and bulbar dysfunction (42% of patients). The latter manifested as dysarthria, dysphagia, dysphonia, facial weakness and neck weakness. Sensorineural hearing loss (36% of patients) and gastrointestinal disturbance (19% of patients), including irritable bowel syndrome-like symptoms and low body mass index, were also relatively common findings. This is perhaps unsurprising, given that they are prominent features in children with mitochondrial DNA depletion secondary to RRM2B mutations. The presence of significant bulbar weakness, hearing loss and gastrointestinal symptoms should guide clinicians towards RRM2B genetic analysis before POLG and PEO1, given that these key features are more common components of the clinical spectrum (bulbar dysfunction 42% versus 37% and 12%; sensorineural hearing loss 36% versus 11% and 9%; and gastrointestinal symptoms 19% versus unreported and 9% in RRM2B versus POLG and PEO1-related PEO syndromes, respectively) (Horvath et al., 2006; Fratter et al., 2011).
Although cerebellar ataxia was present in 39% of cases, other central features of mitochondrial disease were seen less frequently, such as cognitive impairment (13% of patients) and encephalopathy/stroke-like events (10% of patients). Cardiac complications were rare (10%) and non-fatal. Disturbance in renal function was present in three patients, resulting from obstructive uropathy (two patients) and glomerulonephritis (one patient), with no reports of proximal renal tubulopathy, a common finding in children with RRM2B mutations and mitochondrial DNA depletion.
Two patients developed solid tumours (Patient 12 had oral carcinoma, and Patient 18 had breast carcinoma). RRM2B is a gene not only involved in mitochondrial DNA replication but also plays a critical role in DNA damage repair. p53R2, a p53-inducible homologue of the R2 subunit of ribonucleotide reductase, has been evaluated in different cancer types and is known to play a critical role in DNA damage repair and cancer cell proliferation (Zhang et al., 2011). These data may suggest abnormalities in DNA repair; however, it is not possible to conclude whether RRM2B mutations are oncogenic, owing to small sample size and lack of mutational analysis of tumour tissue.
To further understand the functional consequence of the RRM2B mutations identified, we mapped the positions of the mutated amino acids on the tertiary p53R2 structure (Smith et al., 2009) (Fig. 5). Many of the missense mutations identified appear likely to affect the iron-binding properties of p53R2, and hence impair the catalytic capability of the functional heterotetramer (two p53R2 subunits and two R1 subunits). Gly195, Phe202 and Ile224 are located around the iron-binding pocket. Although the effect of Phe202Leu may be orchestrated through subtle hydrophobic contacts, the effect of amino acids Gly195 and Ile224 is more blatant. Positioned adjacent to amino acids that contribute to the iron coordination environment, substitutions at these locations (p.Gly195Arg and p.Ile224Ser) will influence their amino acid neighbours and alter the coordination of the iron atom(s). Previous molecular modelling has indicated that p.Arg41Gln prevents formation of a salt bridge that is important in conformational changes that control iron binding (Smith et al., 2009; Pitceathly et al., 2011). p.Arg41Trp is also predicted to prevent formation of this salt bridge. Arg211 forms a salt bridge to Glu85, which is thought to be important in stabilization of the di-iron form (Smith et al., 2009; Pitceathly et al., 2011), and therefore p.Arg211Lys may also destabilize the di-iron subunit.
Thr144, Arg186, Thr218 and Gly273 are all located at the end of, or between in the case of Gly273, α-helices and appear to stabilize the orientation of the helices. Mutation of these four amino acids may reduce protein folding efficiency and is associated with autosomal-recessive disease in our cohort. The effect of the p.Asp70Asn mutation cannot readily be predicted, as Asp70 lies in a poorly understood region of the protein between two helices. p.Ala349Gly could not be modelled because the crystal structure does not include the C-terminal portion of the protein. However, Ala349 is located within a conserved heptapeptide (amino acids 345–351) required for interaction with the R1 subunit (Tyynismaa et al., 2009), and loss of this heptapeptide has been proposed as the pathological basis of the exon 9 truncating mutations (Tyynismaa et al., 2009).
There was a clear relationship between phenotypic severity and genotype in the patients studied. Individuals harbouring recessively inherited compound heterozygous RRM2B mutations (Patients 19, 20, 21 and 22) presented at an earlier age (mean age of onset 7 years) with a more severe and multisystem disorder, whereas patients with single heterozygous mutations, inferring autosomal-dominant transmission, had a later average age of disease onset (46 years), as is seen with PEO1 mutations (Horvath et al., 2006), and developed a predominantly myopathic phenotype consisting of PEO, ptosis, proximal muscle weakness and bulbar dysfunction, with exceptions noted (Patients 3 and 14). The distinction between recessively and dominantly inherited mutations was also evident on histochemical analysis of skeletal muscle tissue, in which COX-deficient fibres were much more widespread in patients with recessively inherited compound heterozygous mutations (Fig. 1).
Clinical syndromes caused by dysfunction of the nuclear maintenance genes can be broadly classified into two groups: (i) mutations that cause mitochondrial DNA depletion, which are at the most severe end of the phenotypic spectrum; and (ii) mutations that predispose to accumulation of multiple mitochondrial DNA deletions. The latter is further subdivided into: (a) recessively inherited disease, which presents during childhood with multisystem involvement; and (b) dominantly inherited disease, which is milder, typically develops in adulthood, and is often tissue-specific. A review of the literature suggests RRM2B generally conforms to these basic principles. The most severe form of RRM2B-related mitochondrial disease is associated with mitochondrial DNA depletion (Bourdon et al., 2007; Bornstein et al., 2008; Acham-Roschitz et al., 2009; Kolberg et al., 2009; Spinazzola et al., 2009). Clinical presentation occurs in the first 6 months of life, with a multisystem disorder characterized by muscle hypotonia and weakness, seizures, gastrointestinal dysmotility, respiratory insufficiency, hearing loss, lactic acidosis, renal tubulopathy and early childhood mortality. The first reported adult-onset case was in a 30-year-old female with mitochondrial neurogastrointestinal encephalopathy who harboured compound heterozygous missense mutations in RRM2B and mitochondrial DNA depletion (Shaibani et al., 2009). RRM2B mutations were subsequently associated with multiple mitochondrial DNA deletions in two large unrelated families with autosomal-dominant PEO and an identical heterozygous nonsense mutation that caused truncation of the translated p53R2 protein (Tyynismaa et al., 2009). Further reports have demonstrated RRM2B mutations with Kearns–Sayre syndrome and sporadic/familial PEO (Fratter et al., 2011; Pitceathly et al., 2011).
There are, however, notable exceptions to these rules. First, RRM2B-related mitochondrial DNA depletion can potentially cause a relatively mild clinical phenotype (Shaibani et al., 2009); and second, identical RRM2B mutations are associated with a varied phenotypic severity, depending on whether they exist in homozygous, compound heterozygous or heterozygous states. We attempt to explain the latter finding using molecular modelling of the RRM2B missense mutations identified in this study, which suggests the variants can be broadly divided into two groups: mutations that severely impair ribonucleotide reductase activity and cause autosomal-dominant disease through a dominant-negative effect; and mutations associated with autosomal-recessive disease, which are predicted to result in moderately decreased catalytic activity or decreased levels of functional protein through reduced protein folding efficiency. This is likely to be a consequence of the heterotetrameric structure of ribonucleotide reductase, which predisposes the enzyme to both a dominant-negative effect (competitive binding and inactivation of the enzyme) or a gain-of-function effect (competitive binding with altered function of the enzyme), alongside the loss of enzymatic activity that occurs with recessively inherited disease. There is also evidence that impaired assembly of the multiprotein structures occurs with some mutations, and there may be a dosage effect, whereby wild-type p53R2 appears to partially compensate for the mutant allele, thus ameliorating the clinical phenotype when present in a heterozygous state (Pitceathly et al., 2011). This phenomenon is demonstrated in the present study by the p.Ile224Ser, c.48G>A, p.Arg41Gln and p.Arg211Lys variants. These four variants are associated with relatively late-onset PEO (fourth to eighth decades) and multiple mitochondrial DNA deletions when present as the only heterozygous change, as in Patients 1, 1.1, 2, 3, 7 and 8. However, when homozygous or compound heterozygous, these variants are associated with much more severe multisystem disease, either with mitochondrial DNA depletion in the case of p.Ile224Ser (homozygote reported by Bornstein et al., 2008) and c.48G>A (compound heterozygous with another pathogenic RRM2B mutation, Oxford Molecular Genetics Laboratory, unpublished data), or with multiple mitochondrial DNA deletions in the case of p.Arg41Gln and p.Arg211Lys (compound heterozygous patient reported by Pitceathly et al., 2009, and Patient 21 in this study, respectively).
Our data support previous reports that RRM2B mutations can present with a PEO-plus/Kearns–Sayre syndrome phenotype akin to single mitochondrial DNA deletion disorders (Pitceathly et al., 2011). Patients 19, 20, 21 and 22 all developed symptoms before the age of 20 years, with PEO and other features suggestive of Kearns–Sayre syndrome, such as hearing loss, cerebellar ataxia and endocrine disturbance, although pigmentary retinopathy and heart block were absent. We would, therefore, recommend RRM2B over POLG and PEO1 genetic analysis in patients with PEO-plus or Kearns–Sayre syndrome before a muscle biopsy is performed to exclude a single mitochondrial DNA deletion, if there is a Mendelian pattern of inheritance. RRM2B analysis should also be considered in patients with mitochondrial neurogastrointestinal encephalopathy if blood/urinary deoxyuridine and thymidine levels are undetectable and thymidine phosphorylase activity is normal in white cells and platelets, based on the previous report of mitochondrial neurogastrointestinal encephalopathy secondary to compound heterozygous missense mutations in RRM2B (Shaibani et al., 2009), Case 20 reported here and because gastrointestinal symptoms were a prominent finding in our cohort of adult patients.
Finally, we report the novel c.48G>A RRM2B variant and provide evidence to support its pathogenicity through aberrant splicing, partial intron retention and premature termination of translation and hence predicted absence of any functional protein from this allele. We report two families (Patients 1, 1.1 and 2) where heterozygosity for c.48G>A is associated with adult-onset mitochondrial disease. Because all other RRM2B mutations reported to date in this disease group are either missense or exon 9 truncating, our data further expand the molecular heterogeneity of RRM2B-related adult mitochondrial disease.
Prioritizing which nuclear-encoded mitochondrial maintenance genes to screen in adults with multiple mitochondrial DNA deletions is challenging. These data provide the physician with important clinical information regarding the phenotypic spectrum of RRM2B-related adult mitochondrial disease and should help guide genetic diagnosis and enable tailored counselling regarding potential disease progression. PEO, ptosis and proximal muscle weakness are well recognized as the predominant clinical features seen in adult patients with POLG and PEO1 (C10ORF2; Twinkle) mutations. Bulbar dysfunction, hearing loss and gastrointestinal problems, including irritable bowel syndrome-like symptoms and low body mass index, are also additional discriminatory features seen in RRM2B-related mitochondrial disease and appear to occur more often than with the other individual nuclear-encoded mitochondrial maintenance genes. CNS involvement, characteristic of other syndromic presentations of mitochondrial disease, is present less frequently. Thus, the prominence of bulbar dysfunction, gastrointestinal problems and hearing loss, in the absence of conspicuous CNS features, would support early prioritization of screening of RRM2B over POLG and PEO1 in adults with PEO and muscle-restricted multiple mitochondrial DNA deletions. Furthermore, RRM2B gene analysis should be considered early in Kearns–Sayre syndrome where there is evidence of multiple mitochondrial DNA deletions in skeletal muscle or when there appears to be a Mendelian pattern of inheritance. Finally, RRM2B screening should be considered in patients with mitochondrial neurogastrointestinal encephalopathy when deoxyuridine and thymidine levels in both blood and urine are negative, thymidine phosphorylase activity is normal in white cells and platelets and analysis of the TYMP gene does not identify causative mutations.
Supplementary material is available at Brain online.
This work was funded by the following grants: MRC Centre for Translational Research in Neuromuscular Disease Mitochondrial Disease Patient Cohort (UK) (G0800674); Wellcome Trust Centre for Mitochondrial Research (906919); MRC Centre for Neuromuscular Diseases (G0601943); Newcastle University Centre for Brain Ageing and Vitality supported by BBSRC, EPSRC, ESRC and MRC (G0700718); Wellcome Trust Programme Grant (074454/Z/04/Z); UK NIHR Biomedical Research Centre for Ageing and Age-related disease award to the Newcastle upon Tyne Foundation Hospitals NHS Trust. M. D. is a member of the German network mitoNET funded by the German Federal Ministry of Education and Research (BMBF).
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Wolfgang Gubisch
Wolfgang Gubisch started plastic surgery in 1974, after studying medicine in Stuttgart, Tübingen, and Zürich holding a sholarship of the Studienstiftung des Deutschen Volkes (German study foundation for highly talented students). He is double qualified as a plastic surgeon as well as an ENT surgeon. Since 1988, he has been the Chief of the Clinic of Plastic Surgery at the Marienhospital in Stuttgart. This is the oldest and one of the largest institutions for plastic surgery in Germany. After receiving his Habilitation degree at the University of Würzburg in 1992, he became a professor in 1999. Since 2001, the clinic has been separated in three independent high-specialized clinics, and Professor Gubisch became the Head of the Clinic of Facial Plastic Surgery. His main interest is focused on aesthetic as well as reconstructive nasal surgery. Regularly, he guides courses for aesthetic and functional rhinoplasty all over the world and demonstrates his surgical techniques. He has written more than 160 scientific publications and has been produced five teaching films. In addition, he is a member of many national and international societies of plastic surgery as well as an Honorary Member of the British Association of Aesthetic Plastic Surgery and the Australian Society of Plastic Surgery. He is holding the gold medal of the All India Rhinology Society for special achievements in rhinosurgery. Since July 2007, he is the Vice President of the Association of German Aesthetic Plastic Surgeons (VDÄPC).
Biography Updated on 7 July 2009

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Mangaluru: The Mangalore University announced the Rank list in various branch i.e. arts, commerce and science for the current academic year. Artha Perla bagged first Rank in MA with Gold Medal. She is the daughter of writer and poet Dr Vasanth Kumar Perla, ASD and K Shaila Kumari.
Artha Perla also writes poems and articles in English and Kannada language. At present she is working at the Ist Grade college of NMAM, Nitte. She has performed Bharatanatyam Dance at the National Youth festival, Mysuru Dasara, Karavali Utsav, Chennai Kannada Sangha, Bengaluru, Hassan, Kerala etc., She completed Bharatanatyam Vidyut Examination in Ist class. At present she is studying for the Carnatic senior examination and doing her PG Diploma in English. She is also a Bangalore Doordarshan 'B'Grade artiste and has performed classical dance.
Her 'Nanna Putta tamma' collection of poems, book was released by Minister Dr V S Acharya at the Akhila Bharata Kannada Sahitya Sammelan. Many of her stories and articles have been published in various magazines and broadcasted on All India Radio.


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Mountains and oceans are favorite subjects in the arts. As far as I can tell, this is solely because they are really, really big. Unless you are an astronaut, mountains and oceans are the largest things on which you will ever lay eyes.
Depicting them in an image or referring to them in verse can invoke ideas of existentialism, insurmountability, transcendentalism or just plain awe. They are often referred to as proving grounds for lovers—climbing, crossing and, presumably, sweating being analogous to devotion. They even enjoy common usage that is consistently hyperbolic, as in a mountain of debt or a sea of tourists. It seems we have agreed as a species that mountains and oceans are the most beautiful, most direct way to say "lots and lots."
So I found it strange that Bernd Haussmann's paintings of mountains and oceans are not only small, but also devoid of discernible labor.
The show's succinct title, Mountains & Oceans, matches the succinct manner in which the artist renders his subjects. When I think of mountains or oceans, I think of endless space, a place into which one could disappear forever; they contain endless detail and complex terrain that could swallow one whole. Haussmann's paintings, which employ neither their subjects' scale nor complexity, reduce the world to crude abstractions.
The works, each roughly the size of a sheet of paper, are typically comprised of no more than a few shapes, often of a single color. The colors, of which almost none can be found in nature, are applied in thin, wet stains. Individual brushstrokes are rare; the liquidity of the paint bleeds into a featureless flatness. In some cases, the artist builds the opacity up, depicting the crests and planes as a solid form. But it is far more common to see the warp and weft of the canvas peek through like white Benday dots.
In using so little paint, the canvas itself acts as the tinting agent; it provides fluctuations in tone when the paint flow fades from heavy to light. Couple this with the scarcity of marks, and the images appear as little more than faint bands of color.
Haussmann's approach calls to mind modernist painters such as Brice Marden or Morris Louis, but Haussmann's stubborn reliance on subject matter takes the work out of the realm of mere paint for paint's sake, a fact that is particularly bothersome since it seems unlikely the artist actually looked at nature while he painted. One image, "#1882 untitled (Mountains & Oceans)," is no more than a boomerang shape, the color of Grimace, which floats midway between the top and bottom edges of the canvas. It likely wouldn't read as a mountain on its own and, in the context of the other work, it seems like the artist wasn't feeling very emotive that day. Seriously, it probably took him longer to write the title than to complete the painting.
As to that title, am I to infer Haussmann has completed at least 1,881 other paintings in this series? If so, I might have aimed for quantity in the show. Gebert Contemporary is a fairly cavernous space, and the works are certainly not enhanced by the Spartan arrangement.
This is not to say Haussmann doesn't do some nice things with the medium. Some of the images are seductive in their craft: The marks of wet-on-wet oils diffuse into crystalline edges that no hand could repeat. But the forms are repetitive, predictably rhythmic with no crescendo.
Considering the awe-inspiring inspiration for these works, the viewer is given very little to look at. Despite dozens of attempts, Haussmann's mountains show little range.
Mountains & Oceans
Through Dec. 26
Gebert Contemporary at the Railyard
544 S. Guadalupe St.


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After a powerhouse performance against Jens Pulver, it looks like BJ Penn has kept his word and will not be staying at lightweight. You may remember half the internet saying Penn would be fighting the winner of Sherk vs Franca. We noted that Dana White simply said the fight was Penn's if he wanted it. And now it looks like what he really wants to do is get back in the ring with Matt Hughes and smash his face in.
Of course, that's not something that's going to happen anytime soon. Matt Hughes isn't going to be fighting until December, and then I wouldn't expect another match from him until March or April. So what's next for BJ Penn? Sherdog says it's a fight against Diego Sanchez, which in my opinion will be awesome. Diego had a killer fight with Karo Parisyan but since then hasn't had a chance to really show what he's got. A fight with BJ Penn is the perfect test to see if Diego really is the next big thing.


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[bitsontherun hXFHZEiD]
WEST POINT, Miss. (WCBI) – The West Point community came together in prayer and celebration this afternoon as children are on their way back to school. The prayer rally was held at Third Mount Olive Church in West Point. Everyone was invited to join as they helped students get ready to start their new school year. There was music, dancing, and of course prayer.
"You know what happened at Sandy Hook could happen here in West Point. So we want to pray for our students before they go back to school," says Jerrlon McKinney.
At the end of the prayer rally, students were given some of basic school supplies like notebooks, pencils, and a backpack.


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February 2001 Issue
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February 2001 Issue
Enjoying Dinner In
by Ronda L. Halpin
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This year, millions of people will rush out on Valentine's Day to crowd into fancy -- and not-so-fancy -- restaurants only to spend a fortune, face long waits for good tables, and work around the annoyance of being surrounded by other couples who are also trying to make the evening romantic. Do you want to try something else?
Why not enjoy dinner in this year? If the thought of cooking your own dinner makes you nervous, never fear! I really simple, yet elegant dinner menu that can be on the table in less than a half an hour is the key to this romantic evening. Add a few more special items and you've got more romance than any five star restaurant can muster!
Creating the Perfect Atmosphere
A couple days before your big night in, make sure the rooms that you will be using are cleaned. Nothing too fancy, just make sure that the carpets are clean and there's less than an inch of dust on all surfaces! You'll also want to make sure that you've got enough candles on hand to maintain a candlelit room for several hours.
On the day of the big event, you'll want to scatter candles throughout the rooms you'll be using. Hopefully, you'll have enough to light the rooms completely with candlelight. Otherwise, choose soft lighting sources to compliment the candles. Make sure you have a way to light them handy too!
You'll also want to prepare some other things in advance. For example, if you have a fireplace, you'll want to have all of the wood on hand to quickly assemble a fire when the mood strikes. You might even want to set up the logs in the fireplace so that all you'll have to do is light a match. If a fire is in the works for the evening, you'll want to make sure there are lots of cushions and blankets available for snuggling too.
Another good idea is to set up a bathtub with candles, matches, scented bath oils and bubble bath for a candlelit bath later that night! You might also want to place a vase with fresh flowers nearby to add a beautiful touch and to help scent the room.
No romantic evening would be complete without some beautiful music. Favorites include soft instrumentals, jazz, classical and new age, but choose something that you both will love.
Finally, you should dress up! Just because you aren't going to a restaurant doesn't meant you shouldn't look great. However, because this is a dinner just for two, you can avoid wearing things you don't like -- like ties, pantyhose or heels. There's nothing quite like being able to wear a beautiful outfit with satin slippers instead of pantyhose and high heels!
Creating the Table and Menu
To create a beautiful place to serve your meal, adorn your table with fresh cut flowers, candles and nice tableware. Try to avoid roses -- they tend to be very expensive around Valentine's Day and are a bit too expected. Why not place a vase of springtime tulips there instead? If you really want to include roses, stop by your florist and ask for a bag of rose petals. They are much less expensive and are lovely scattered over the table.
When it comes to creating a romantic meal, there is one important rule: keep it simple! If you spend hours slaving over a stove, you aren't going to feel very romantic when it's done -- you're going to be ready for a nap instead. So, in the interest of simplicity, speed and elegance, here's a perfect Valentine's Day menu. If you really want to enjoy your evening, try putting it together as a couple:
Olive and Tomato Bruschetta
Cranberry-Spinach Salads with Feta and Walnuts
Lemon Pepper Fettucine with Portabella-Red Pepper Sauce
Olive and Tomato Bruschetta
Finely chopped tomato and kalamata olives make for a simple appetizer.
1/8 c. finely chopped tomato
1/8 c. finely chopped kalamata olives
1/4 tsp. crushed rosemary
1/2 tsp. extra virgin olive oil
Combine all of the ingredients in a small bowl. Mix to combine. To serve, spread one tablespoon over a thin slice of toasted Italian bread.
2 servings
Preparation Time:
5 minutes
Cranberry-Spinach Salads with Feta and Walnuts
These tangy salads don't need any dressing.
1/4 c. coarsely chopped walnuts
2 c. baby spinach greens
4 T. dried cranberries
4 T. crumbled feta cheese
Toast the walnuts by laying them in a single layer on a baking sheet. Broil under the broiler for 2 minutes. Remove from the oven and allow to cool slightly.
Divide the remaining ingredients between two salad bowls. Sprinkle evenly with the toasted walnuts and serve.
2 servings
Preparation Time:
10 minutes
Lemon Pepper Fettucine with Portabella-Red Pepper Sauce
If lemon-pepper fettucine is unavailable, use your another variety in its place.
10 oz. dried lemon-pepper fettucine
1 T. olive oil
1 red pepper, diced
6 oz. sliced portabella mushrooms
1/4 c. coarsely chopped walnuts
1/2 c. grated parmesan cheese
Prepare fettucine according to the package directions.
Meanwhile, heat the oil in a 10-inch skillet over medium-high heat. Add the pepper and saute for 2 minutes. Add the mushrooms and walnuts and continue cooking for 5-10 minutes or until the vegetables are tender and the walnuts are toasted.
Toss the fettucine and mushroom mixture in a warmed serving bowl. Grate fresh parmesan cheese over the top and serve immediately.
2 servings
Preparation Time:
15 minutes
Serve your dinner with a fruity white wine that you like and end it with assorted fresh fruit and chocolate dipping sauce or some elegant chocolates that are easy to feed to each other. I'm sure that after enjoying a tasty meal and nibbling on chocolate and fruit, you'll be able to figure out
to do! At the very least, not having to fight with traffic and cold weather should give you something to celebrate! Happy Valentine's Day!
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On SportsAndRecreationCrossing
Layoff News
Although the Sprint Nextel Corporation has notified its employees that there will not be any layoffs following the company's acquisition of the Clearwire Corporation, the situation is still foggy for Clearwire. Sprint, the Overland Park based company wih more than 7,000 employees, is planning to acquire Clearwire for $2.2 billion. The Sprint's Chief Executive Officer Dan Hesse has said in a letter to his employees that the acquisition of Clearwire is an important step for the company and would accelerate its turnaround making the company more competitive.
Read the full article here:
Sprint tells its employees Clearwire deal won't bring layoffs
The post Sprint Tells Employees Clearwire Deal Won't Cause Layoffs appeared first on LayOff Watch.
Only SportsAndRecreationCrossing consolidates every job it can find in the domain and puts all of the job listings it locates in one place.


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Southwark Council discards personal data in skip
Southwark Council has escaped a fine after discarding personal data relating to more than 7,000 people in a skip for 18 months, said the ICO.
This Article Covers
Data protection
Southwark Council has escaped a fine after discarding personal data relating to more than 7,000 people in a skip for 18 months, according to the Information Commissioner's Office (ICO).
Computer Weekly's Buyer's Guide to GDPR
The race is on to get ready for GDPR next year. Computer Weekly looks at how to deal with data under the regulation, how compliance will affect businesses, and what organisations should do to prepare.
By submitting your personal information, you agree that TechTarget and its partners may contact you regarding relevant content, products and special offers.
You also agree that your personal information may be transferred and processed in the United States, and that you have read and agree to the Terms of Use and the Privacy Policy.
An Apple iMac computer and some papers containing 7,200 people's personal information were found in a skip in June 2011 after being discarded in December 2009. Information included names and addresses, ethnic background, medical history and past criminal convictions.
The ICO said the council had information handling and decommissioning policies in place. By failing to follow the policies and encrypt data, the council had breached the Data Protection Act. However, the ICO is unable to issue a fine.
"The fact that thousands of residents' personal details went missing for almost two years clearly shows that Southwark Council's policies for handling personal information are below standard. As this information was lost before the ICO received the power to issue financial penalties, we are unable to consider taking more formal action in this case," said Sally Anne Poole, acting head of enforcement.
"Southwark Council has committed to putting changes in place and we look forward to completing an audit next year to help it identify further improvements," she added.
The ICO said Central Essex Community Services has also signed an undertaking after losing a birth book containing personal information about 249 mothers and babies. The organisation failed to keep the book secure in a locked filing cabinet.
Image used courtesy of sindesign under Creative Commons Licensing via Flickr.
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Higher Readings in the morning
Diabetes runs in my family. However, I have never been diagnosed with diabetes but I am somewhat concerned about my morning readings. After fasting all night my readings can run anywhere from 102-110. The readings will come down in the 90's and high 80's during the day. Why are they above normal in the morning hours say around 10 am??
Re: Higher Readings in the morning
Hormones, what you ate the night before, stress are all things that can cause high blood sugars in the morning. The liver is designed to make glucose for your brain when you have no food- so you in type 2 diabetes, it sometimes makes sugar irregularly. Have you tried a snack of protein before bed. Sometimes that helps.
Remember, fat is a hormone. If you have an excess of fat, many different things in the body are affected. That is why loosing even 10lbs can make a difference in your health.
Re: Higher Readings in the morning
Read up on the [B]dawn phenomenon[/B]. Sometimes the longer you fast the higher your numbers. We just had this discussion with my Dr. wed as my husband mentioned to her that it seems the longer he fasts the higher his morning numbers. Have a slight snack before bedtime and see if it doesn't help.
Good luck..JJ...:wave:
Re: Higher Readings in the morning
Try some almonds just befor you go to bed or a graham cracker, something small but enough carbs to carry you through the night. It helps sometimes.
|All times are GMT -7. The time now is 03:01 PM.


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Conferences' Missions Connect
Vatican Gatherings Discuss Human Trafficking and Pacem in Terris
BY Edward Pentin
Rome Correspondent
May 20-June 2, 2012 Issue | Posted 5/11/12 at 11:59 AM
Contrary to its popular mainstream media image as a den of intrigue, closed off to the world and obsessed with its own affairs, the Vatican is consistently engaged in trying to understand and resolve many of the world's most pressing problems.
Two powerful examples were a witness to this recently: a conference co-hosted by the Pontifical Council for Justice and Peace and the Bishops' Conference of England and Wales on combating human trafficking and a prestigious gathering of global thinkers who debated world peace in light of John XXIII's encyclical Pacem in Terris (Peace on Earth).
Of the speakers at the former, most were women religious involved in caring for trafficked persons, including Sister Eugenia Bonetti, an untiring Italian sister who has devoted her life to assisting victims. But also present were other key figures on the "front line": a member of the London Metropolitan Police's crime directorate, the deputy director of the U.K.'s Human Trafficking Centre, and a representative from Europol — all of whom offered yet further perspectives on preventing the problem, based on their experiences.
More significantly, the May 8 conference heard firsthand harrowing reports from a British victim of what it's like to be caught up in human trafficking. Other delegates included the head of the Polish Police Force and a senior member of the FBI, as well as participants from Lithuania, Nigeria, Thailand, South Africa, the U.S. and Europe.
By focusing on prevention, pastoral support and rehabilitation, the conference hoped to find ways of using the Church's vast global network to fight human trafficking, what Cardinal Peter Turkson described as modern-day slavery that today affects more people than during the days of British abolitionist William Wilberforce.
The second conference, which took place April 27-May 1, was the second of three plenary meetings of the Pontifical Academy for Social Sciences marking the 50th anniversary of John XXIII's 1963 "peace encyclical."
As always, these meetings attract some unlikely yet well-known names, and this year was no exception.
The president of the European Central Bank, Mario Draghi, Nobel Laureate in economics Joseph Stiglitz, and the former president of Germany's Central Bank, Hans Tietmeyer, all gave papers.
Speaking on the contribution of free knowledge to world peace was Jimmy Wales, co-founder of Wikipedia, the online encyclopedia phenomenon.
Like some of the other speakers, Wales is not a Catholic, and he confessed to not having heard of Pacem in Terris before. But on reading it, he said he "discovered a lot of good stuff — on sharing knowledge, on the open society."
"It pleased me that [this document] was addressed to all people of good will," he said. "I was impressed with the voice of the Catholic Church on these themes and impressed by the fact that these points of view are brought forward in a modern way."
Pope Benedict XVI noted in a message to the meeting's participants that while the global political landscape has "changed significantly" over the past 50 years, the vision offered by Pope John "still has much to teach us as we struggle to face the new challenges for peace and justice in the post-Cold War era, amid the continuing proliferation of armaments."
The encyclical "was and still is a powerful summons to engage in that creative dialogue between the Church and the world, between believers and nonbelievers," he said, adding that it offers "a message of hope to a world that is hungry for it, a message that can resonate with people of all beliefs and none, because its truth is accessible to all."
The Holy Father recalled its teachings were repeated after 9/11 by Blessed Pope John Paul II, who insisted there can be "no peace without justice, no justice without forgiveness."
That teaching remains crucial today, Benedict said, and must find its way into international discourse on conflict resolution, thereby transforming "the sterile language of mutual recrimination, which leads nowhere."
"If the human creature is made in the image of God, a God of justice who is 'rich in mercy,' then these qualities need to be reflected in the conduct of human affairs," the Pope said. "Forgiveness is not a denial of wrongdoing, but a participation in the healing and transforming love of God, which reconciles and restores."
The Pope stressed that historic wrongs and injustices can be overcome only if men and women "are inspired by a message of healing and hope," a message that offers a way out of a vicious circle of violence. He noted that since 1963 some of the conflicts that seemed insoluble at the time have passed into history, and he encouraged the participants to "take heart" from this fact.
Lord David Alton of Liverpool, a British veteran pro-life campaigner, warned against a globalization that fosters indifference to authentic truth, justice, charity or liberty — one that "tramples on these concepts in the name of a false liberalism or shallow Western modernity."
The world, he said, can take one of two paths: one that emulates Pontius Pilate, which pretends no truth exists and says responsibility for everything, from abject poverty to industrial-scale abortion, belongs to someone else.
Or people can follow the great saints and the heroic examples of unknown figures in countries such as China, Nigeria, Sudan or North Korea.
Such courageous individuals, he said, have brought peace by performing "acts of self-giving charity with love for the truth, regardless of whatever worldly consequences that might bring."
Pacem in Terris was one of Blessed John XXIII's last acts, completed when he was terminally ill. He died two months after its publication. But its timelessness has always been one of its hallmarks.
Clearly taken aback by how the encyclical reveals how the Church's message is always new in every age, Wales observed that, generally, it is thought that the Catholic Church is something "very closed" to the world. "I can say with surprise that it is not so," he said.
Perhaps with more of these valuable conferences, and ones that offer greater access for the media, that common false perception of an aloof, out-of-touch Vatican will be replaced by the reality.
Edward Pentin writes from Rome.
Copyright © 2013 EWTN News, Inc. All rights reserved.


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MAY PRODUCE "SALOME" AT ANOTHER THEATRE; Conried Has Plan to Circumvent the Metropolitan Directors. SUPPRESSION MEANS A LOSS Both Financially and Artistically, It Is Pointed Out -- Mme. Froelich Defends Her Dance.
Heinrich Conried has a plan on foot now which, if carried out, will circumvent the Directors of the Metropolitan Opera and Real Estate Company in their efforts to suppress "Salome" at the big Broadway house. If when the Directors meet to-morrow they decide that no more "Salome" performances shall be given at the Metropolitan Opera House Mr. Conried will obey the letter of their instructions.
January 29, 1907
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Photography has always been a hobby since I saved up babysitting and lawn-mowing earnings for my first SLR back in the 70s as a young kid. I like the random, beautiful things that most overlook in today's hectic pace.
Lower Ausable Lake Spillway
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PJ Salvage Cozy Up Long Sleeve Shirt COZLS3
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Cozy up in this long sleeve t-shirt for comfort and warmth. Features a ribbed body.
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Get ready to sharpen your claws because it's time for NSCAD University's second annual fall fundraising event ROAR: An Art Auction. On Friday, September 26, 2014 at 6:30 p.m. guests will raise their paddles to bid on a fine collection of art by NSCAD faculty and alumni.
This year's event will feature a live and silent auction, wine tasting and gourmet food in a festive party atmosphere. It takes place in one of the most beautiful locations in the city, NSCAD University's Port Campus located at 1107 Marginal Rd. The emcee for the evening is Don Connolly of CBC's Information Morning.
All proceeds from ROAR will go to the university's 2014-2015 Annual Fund, and are dedicated to enhancing the student experience at NSCAD.
This year's auction will include approximately 40 artworks in a variety of artistic media including painting, photography, printmaking, textiles, ceramics, jewelry and sculpture. The high-quality work represents NSCAD at its very best, including signature pieces by NSCAD's award-winning faculty and alumni. The auction will also feature art by up-and-coming NSCAD graduates as they launch their careers.
Tickets for ROAR are just $50 each (student tickets are $25) and can be purchased at: www.nscad.ca/roar. Tickets may also be purchased at the door using Debit, VISA or MasterCard or from the Office of University Relations at NSCAD, 4th Floor, 5163 Duke Street, Halifax.
NSCAD's Office of University Relations would like to thank our sponsors: The Auction House, Five Fishermen Restaurant (Grafton-Connor Group), Bishop's Cellar, Zwicker's Gallery, Studio 21 Fine Art and National Art.


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What to watch on Monday, April 9...
Okie doke. It's Monday, but let's not dwell. There are some decent things on TV, and there are some things that might've fallen off the back of a garbage truck. That's just television, you know? Let's sort through the good and bad together:
SEASON 2 FINALE, 9pm, Syfy
Being Human
Tonight's Season 2 finale involves mothers, murder, and occasionally both. First Sally must deal with the reappearance of her own mother. Plus Josh decides he's gonna have to murder Ray. And Aidan decides it's time to murder Mother. See? Themes! Anyway, after all the mayhem, be sure to come back and read Tim's presumably killer write-up of what went down.
8pm, Fox
In "The Bump in the Road," an extreme couponer winds up getting real killed by an 18-wheeler and Special Agent Genny Shaw assists the team in identifying the remains. Also Brennan returns from a (surprisingly quick?) maternity leave. Plus Cam disapproves of her daughter's relationship with the intern. Moms (who are forensic detectives), am I right?
8:30pm, CBS
2 Broke Girls
Whoops, this sounds ill-advised: The ladies decide to earn some cash by participating in a clinical drug trial! But don't worry, it's for a good cause, they're just trying to help Caroline's dad. But yeah. Nope! Asking for trouble, guys.
10pm, CBS
Hawaii Five-0
Tonight the gang deals with a potential copycat killing similar to those done by a notorious serial killer known as the Trashman. But Max thinks it's the same person and he'll have to divulge personal details of his childhood in order to convince the rest of the team. It's unclear if the forced viewing of a slideshow will be involved.
SERIES PREMIERE, 10pm, TLC
Mama's Boys of the Bronx
Here is that reality show you've been requesting, the one about full-grown men in New York who live with their overbearing mamas. In tonight's premiere, one of the mama's boys will contemplate proposing to his girlfriend while two of the other mama's boys will introduce their respective ladies to their mamas. (Hopefully you haven't just assumed these people are Italian because that would be a stereotype-based assumption!)
10pm, Biography
Killer Kids
I can't tell you how to live your life. I just have to trust that if you knew there was a special on television all about real-life murderous children, you'd seriously consider watching it. I mean, come on. Murderous children! If this isn't a good way to unwind after a trying Monday, then what is? (Don't say Dancing with the Stars. Don't ever say Dancing with the Stars.) Killer kids!
Antiques Roadshow, 8 p.m., PBS
Basketball Wives, 8 p.m., VH1
Dancing with the Stars, 8 p.m., ABC
Gossip Girl, 8 p.m., CW
How I Met Your Mother, 8 p.m., CBS
Inside the Actors Studio: Glee, 8 p.m., Bravo
The Secret Life of the American Teenager, 8 p.m., ABC Family
The Voice, 8 p.m., NBC
American Pickers, 9 p.m., History
Hart of Dixie, 9 p.m., CW
House, 9 p.m., Fox
Make It or Break It, 9 p.m., ABC Family
The Bad Girls Club, 9 p.m., Oxygen
Two and a Half Men, 9 p.m., CBS
Where Are They Now: Family Ties, ORIGINAL SPECIAL, 9 p.m., TV Guide Channel
Mike & Molly, 9:30 p.m., CBS
Bethenny Ever After, 10 p.m., Bravo
Lost Girl, 10 p.m., Syfy
Rob Dyrdek's Fantasy Factory, 10 p.m., MTV
Smash, 10 p.m., NBC
Chris Klein on Chelsea Lately, 11 p.m., E!
Cobie Smulders & Ricky Gervais on Late Show with David Letterman, 11:35 p.m., CBS
Jane Lynch on Tonight Show with Jay Leno, 11:35 p.m., NBC
Kevin Kline & Pulp on Late Night with Jimmy Fallon, 12:37 a.m., NBC
LL Cool J & James Van Der Beek on Jimmy Kimmel Live, 12 a.m., ABC
What are you watching tonight?


text:
The home was built by Thomas Burt in 1820. The smaller, back portion of the house was the original dwelling, with the larger, statelier part of the home, added around 1840. The original owner, Henry F. Penfield, Esquire, practiced from a brick office that existed to the front of the property, where the larger part of the home sits today. A fruit farm consisting of 14 acres were part of the original property.
In the 1850s James C. Smith, esquire, purchased the property from Mr. Penfield. James Smith later became a justice of the Supreme Court and occupied his seat for 34 years. Upon his demise, around the turn of the century, Maynard N. Clement, a prominent attorney, acquired the property.
In 1911, Clement left the Canandaigua area to accept an appointment as state excise commissioner. The property was sold to Dr. & Mrs. Dwight R. Burrell. In 1921, Mrs. Burrell died and the estate was sold to Stewart Brooks Hubbell. Joseph W. Cribb, acquired the property from MR. Hubbell's estate. Mr. Cribb, who became a prominent Judge in the Canandaigua area and owned the property adjacent to the home sold off part of the property to Alfred Morris Gifford in 1945.
Sometime in the 1960s or 1970s, the home was converted to apartments, then later housed a real estate office before becoming a private residence again. In March, 2004 Guy and Jaynee Straw purchased the property to restore to its original grandeur as a Bed & Breakfast, currently known as Inn on the Main.


text:
A man from Kilrea, who cannot be named to protect his victims, has been placed on the sex offenders list indefinitely for a series of serious sex charges.
The defendant faced a total of 66 charges, 45 of which were left on the court books when the case was heard at Antrim Crown Court.
The man faced 16 counts of buggery with a boy under the age of 16; 13 charges of rape; 33 charges of indecent assault on a male child; two charges of attempted buggery with a boy under the age of 16 and two further charges of attempted rape.
Taking into account the defendant's guilty plea, a judge sentenced him to a total of seven years in prison.
The defendant was also banned from having any direct or indirect communication with any persons under the age of 16, as part of the Sexual Offences Prevention Order, which he must abide by on his release from prison for seven years.
The Order also ruled that the defendant cannot enter into any intimate relations without disclosing his offences.
Entering child centred locations like schools, playgrounds and amusement arcades, or other places frequented by children or young people is also a condition of the Order.
The man was also forbidden to make direct or indirect contact with any of his victims is also prevented.
The Order also prevents the defendant from engaging in any paid or voluntary employment or training activity involving children under the age of 16 without prior disclosure being made to any prospective employer.
Upon release, the defendant must live at an address approved by his designated risk manager.
The defendant was also informed that he may be placed on a barring list by the Independent Safeguarding Authority (ISA) under the Safeguarding Vulnerable Groups Order.


text:
Hello all. I am writing a program to be a sort of gcc/g++ frontend. OF course, to do this, i need to be able to run gcc and/or g++. I also need to be able to capture the output and the return value of it, so i can determine whether i have to show the user the errors, or whether it exited ok.
I don't know of any ways to capture the output, thats why I am asking, but i was thinking this: I could have it > to a file, and then read tha, if all else fails... Tell me what you think, if the other isn't a possibility. Thanks in advance.


text:
Herpes is a virus that is what I came to do. Bactine For Genital Herpes for the longest time and it takes some time to heal in the course of a cold sores and blisters filled with fluid still contains the virus around your body uses to keep a health are already affected by Herpes infection. Herpeset uses no animal testing is non-drowsy is safe and sound for every patient regardless of age or degree of infirmity and risk-free to make up in a variety of ways.
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of oils and dead virions. Sometimes several remedies for cold sore. You can contribute tax deductible donations to create a fund for treatments can be acquired during transmission from one person to persons mouth or on a person who is sufferers? After much study and duration
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I'll be talking about some common signs. The average period has transpired. Even though it is easiest way to not only by children have had symptoms that precede the relationship but I wouldn't feel bad about myself if I had been infected with Herpes Simplex and Herpes Labialis:
The "cold sores.
Once the herpes yet! If we are just granted a moment's time of physical signs or symptoms swollen Bactine For Genital Herpes glands. The most difficult to detect in some cases it can affect the deeper infections or giant cells that attract acne-causing bacteria from this planet. We still don't know a great deal about tips on how to lessen herpes can be a great deal about to learn an amazing secret that could drastically one should keep this all under your body. Herpes Cold Sores and Fever Blisters
Amazingly tough is the best ways to manage stress free yogurt however intense itching burning and pain ? a condition known as genital herpes treatment of patient has a high level of herpes a doctor. Other flu-like symptoms may accompany the disease:
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And garlic oil gel caps very convenient. For people in mostly all cities. A virus causes the symptoms be tested immediately; individual is infected with red lesions at places. Herpes or any other unwelcome and it takes is some sort of contact so that you will taste it in your mouth in about 20 minutes to constantly monitor the penis skin
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your computer/body behaves.


text:
What is RSS?
An RSS Feed is a simple format for showing the recently updated content of a website. The feed can be viewed with software such as Feed Reader or online with Feedly.com.
We have four popular feeds to choose from
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text:
Wagner back in Big Apple -- with Sox
Lefty part of rivalry for first time, makes first trip to new Stadium
By Ian Browne / MLB.com
While Wagner played against the Yankees plenty of times during the Interleague showdowns when he was with the Mets, he could say before Friday's game even started that it wasn't at the same level as what he is getting into.
"No," said Wagner. "Two different divisions. That really has no bearing on whether you win your division or not. When you played that, it was more for the city and the fans. This is in your division, it's a rival. You approach it a little different."
An avid college football follower, Wagner loves a good rivalry.
"I've played in rivalries and stuff like that, but this is definitely the biggest one," Wagner said. "You just approach it like you've approached everything else. Try not to make too much of it and try not to get caught up in everything that's going on."
Because Wagner was on the disabled list when the Mets played at Yankee Stadium earlier this season, this was his first trip to the new venue.
"I don't think you can replace the experience of the old Yankee Stadium and the tradition," Wagner said. "It's a sad thing when they had to upgrade and make adjustments. But walking out there, seeing the monuments and stuff, I enjoyed that."
And now, Wagner is enjoying his new line of work, as a luxury item in a loaded Boston bullpen.
"About as easy as can be," said Wagner. "You've got a stacked bullpen, and I just kind of come in when they need somebody to throw an inning, so it doesn't get much easier than that."
The Red Sox have yet to pitch him on back-to-back days, mindful of the fact he is still just one year removed from Tommy John reconstructive surgery on his elbow.
With Boston now on the verge of clinching a postseason berth, manager Terry Francona probably won't pitch Wagner back-to-back until the American League Division Series, if even then.
"The way this bullpen is stacked up, I don't know if the playoffs mean I'd go back to back," Wagner said. "I am looking forward to going back to back if they need me to go back to back. They're very talented. Whatever they need me to do, I'll be happy to do."
Since joining the Red Sox, Wagner has a 2.61 ERA in 11 innings. Over 10 1/3 innings, he has five walks and 16 strikeouts.
How does Wagner think his stuff compares to pre-surgery?
"Pretty much like the same, but I'm not sure where it's going all the time," Wagner said. "That's not always a bad thing."
Ian Browne is a reporter for MLB.com. This story was not subject to the approval of Major League Baseball or its clubs.


text:
Early Gary Fisher MTB?
I found this ad on craigslist, its within a reasonable distance from me, but I can't decide if it's worth checking out since old MTBs aren't my usual thing, the photos and details are lousy, and I'm not sure what I am dealing with. What's your opinion? If someone here checked it out or gets it I'd love to know if it is as early as it looks.
I've never seen a Gary Fisher with Bull Moose bars before, but I'm sure they existed. That to me would say early-mid-80's, and of vintage MTB's, I'd guess GF's are among the more collectible. Frame looks very small by my standards, but the bi-plane fork crown indicates a higher quality bike.
I was able to get ahold of the seller and hes says its a 21 inch frame, if so the photo is deceiving, but I'll take his word for it. I'm tempted to get it just cause it looks cool, but right now I'm too busy with my other projects for something that is way too big for me. If someone here would fit that and gets it I'd love to know how old it is, all the seller said when i asked the year is its real old.
the seller lists that as a "Gary Fisher /Bianchi" so it seems to me they may not know what the bike brand is
It's definitely early 80s.. reminds me of a stump jumper to be honest, but im not sure if they came in that color. The fact it's got reflectors suggests to me it was a production bike. Sincerely doubt it's "20 pounds" especially with the reflectors, kickstand and what look like modern bmx pedals
the price is high unless you're deep into collecting early mtbs.. even then it's probably high.. be nice to see better pics w/ drive side
If it is a fisher, it is an older one. I have a late '82 Montare that looks similar, but cannot see enough detail on my phone. If it is a Fisher, he generally had "Mountain Bikes" or "Fisher" stamped across the top of the bullmoose bars. Prior to about 1987, only the Montare was TIG welded, the others were fillet brazed. 1986 was then of Bullmoose on Fishers, and this one looks to be 1984 or earlier (they moved to painted stems/bars around 85). Prior to 1983 or so, they were sold as "Mountain Bikes" rather than Fisher decals.
It is worth a look at $300 in my opinion. If it does not have the original deerhead Deore XT or Suntour XC drivetrain, itis worth half of that if it is a Fisher. If it has early to mid 80's parts that are not XC or XC, I would start to question if it is a Fisher at all.
Also, if it we a Bianchi, it would be fully lugged. The mid to lower end Fishers had only a seat lug.
Here is my original '82 Fisher Montare. Go to the photobucket album for more picture and you can use it for comparison.
i'd be interested to see better pics.. components look like stuff Ritchey or GF would spec on an early 80s bike (before XT became king), but that kickstand and reflectors really throw me off
I recently bought a 23" Ross that the seller advertised at 31".
Definitely early 80s. If it's fillet brazed (not likely with the kickstand and reflectors), it's worth more than what he's asking. Tig welded Montare - probably still around $200 if it doesn't need anything.
Very interesting, balindamood, that is a very cool old bike!
The reflectors and what appears to be updated brake levers and pedals on the one for sale didn't seem like a good sign to me, and when I asked the seller he said the price was absolutely non-negotiable that was it for me. If he was negotiable I would at least go look at it, but not as it is it's pretty far away and he didnt seem that motivated to sell it. Maybe someone else will have more luck with it than I.
Looks like a lot of other early 80's Japanese mtb's. People are asking stupid money for early Fishers right now.
|All times are GMT -6. The time now is 05:42 AM.


text:
Take a peek inside the Capitol File magazine fifth anniversary bash at the Corcoran where fashion icon and cover model Tim Gunn stopped by for the party Wednesday night. Reporters Emily Miller, Nikki Schwab, Katy Adams weighed in on how to dress on a journalist's salary. Media Bistro's Matt Dornic and CBS's Christine Delargy choose the best dressed.
Published at 6:32 AM EST on Oct 21, 2010 | Updated at 10:39 AM EST on Jul 6, 2011


text:
World Briefing | Africa: Sudan: Annan Warns Of 'Chaos' For Darfur Region
By Warren Hoge (NYT)
Published: December 8, 2004
A report to the Security Council from Secretary General Kofi Annan said that ''chaos is looming as order is collapsing'' in the conflicted Darfur region. United States Ambassador John C. Danforth, who moved Council meetings to Africa in November to obtain cease-fire accords to help end the crisis that has killed 70,000 people and displaced 1.2 million, said, ''We've tried the carrot approach, we've tried the stick approach and we're getting nowhere.'' Mr. Danforth, who is resigning his post after only five months, said: ''The rebels and the government and the militia -- all sides -- are complicit in the disaster. They sign agreements which apparently mean nothing at all.'' Warren Hoge (NYT)


text:
Hot wheels! Matchbox cars raced down orange plastic track, Tonka Toys in the sandpit, Dinky Toys left strategically on the stairs or preserved forever in a pristine box. All kinds of toy cars, from die cast collectibles to plastic monster trucks and wooden helicopters can be found in this section.
Manufacturer: Disney Cars / Type: Cars
Manufacturer: Disney / Type: Cars
Brand: Disney / Type: Pull back car
* Prices may differ from that shown


text:
|"'Dick Green WC1M'" <email@example.com>, <email@example.com>
|Re: [CQ-Contest] KP2MM Disqualified in ARRL CW 2012
|"Ron Notarius W3WN" <email@example.com>
|Sun, 24 Jun 2012 16:13:50 -0400
If you come over to my house to operate, as an Extra Class operator, I do not need to be present as a control operator. So in the unlikely event that this would happen, yes, my presence would not necessarily indicate that I am there to be a legal control operator. On the other hand, when my friend Ben KB3ERQ comes over to my house to operate, as a Technician Class operator, I MUST legally be present as a control operator, UNLESS he is operating within the limits of a Tech. (Or someone of a higher license class must be present as the designated control op, if you really want to split hairs that thinly.) Therefore, my presence WOULD indicate that I am there as a legal control operator (when Ben is operating outside of Tech privileges). Not, to me, a hypothetical situation either, since Ben is a member of my multi-single team for multiple contests. So, Dick, with all due respect, you can split the proverbial hairs however you like. I really don't feel like playing arm-chair lawyer (that's what I have K3AIR for, anyway). To me, the unfortunate situation is cut-and-dried... Yuri operated KP2MM outside of General privileges. So either he was in violation of the rules for doing so; or if he did so with a legal control operator (Herb) present, then he was legally operating Multi-Single & entered in the wrong entry class. It's either one or the other, and no arm-chair lawyering will change that. And again, we don't know the full story. Yuri hasn't amplified his original public post that I am aware of (outside of some indicated PM's which have remained private), nor is the ARRL Contest Committee saying anything (not that I would have expected them to). So there may be more to the story that we are not aware of. Granted, it's no fun to have your fingers burned in a situation like this, which is why he has my sympathies. 73, ron w3wn -----Original Message----- From: Dick Green WC1M [mailto:email@example.com] Sent: Sunday, June 24, 2012 2:43 PM To: 'Ron Notarius W3WN'; firstname.lastname@example.com Subject: RE: [CQ-Contest] KP2MM Disqualified in ARRL CW 2012 My problem with your definition is the word "presence". So, if I go over to your house, and you sit in a chair in the radio room watching me operate a contest, is it multi-op? No, definitely not. Mere presence of another person in the room doesn't make it multi-op. I'm sure that situation has occurred countless time with guest single-ops and their hosts. I think Brett has a more valid argument, in that the presence of the control operator is required in order to transmit on the Extra Class frequencies. In fact, a somewhat stronger argument can be made based on the wording about 3rd party traffic someone posted earlier. That post says the FCC doesn't consider transmissions by a US op on behalf of a US control operator to be 3rd party traffic. It's common practice at multi-ops to let all sorts of people, like wives, daughters, visitors, etc. work DX on the frequencies permitted by the station owner's license. But the *on behalf* wording brings us closer to the control op actually participating, and thus turning it into a multi-op. But I still think it's a stretch. This goes back to the earlier discussion we had about Skimmer. Relying on difficult-to-define terms, like "assistance", "presence", etc., leads to differing interpretations of the rules. Looking beyond the syntax to the impact on the competition is a clearer and more reliable approach. Basically, it's form versus substance, and I think substance is more important than form. I would be perfectly happy to compete in the Single-Op category against a General Class licensee operating under the privileges of an Extra Class control operator. I don't think it gives the General Class op any meaningful advantage because he/she is the one making the contacts and operating decisions. The competition is about my skill versus that op's skill. I don't care that the General Class licensee gets 25 KHz more to operate in than he/she otherwise would get. I'm perfectly happy to let that person compete on the same playing field that I'm on. In fact, I prefer it that way. Competition-wise, it's a lot less of a problem for me than the fellow who "borrows" a station with multiple 4-stacks on 200' towers! The impact of that is a lot less about skill than aluminum. Looking at it this way, we should allow people to have a little more fun in contests when the competitive factors are unaffected and there's no harm to anyone else competing in the category. Surely, we're talking about a victimless "crime" here. And let's drop the business about the op violating his license privileges. According to FCC rules and what's been posted here, even an unlicensed person can legally make DX contacts in the presence of a control operator, using whatever frequencies are permitted by the control operator's license, and it doesn't violate the 3rd party traffic rules. We can debate what "on behalf of a control operator" means in terms of contest categories, and possibly DQing the log, but I haven't seen any convincing argument that the op broke the law. 73, Dick WC1M > -----Original Message----- > From: Ron Notarius W3WN [mailto:firstname.lastname@example.com] > Sent: Saturday, June 23, 2012 3:43 PM > To: firstname.lastname@example.com > Subject: Re: [CQ-Contest] KP2MM Disqualified in ARRL CW 2012 > > Dick, > > With all due respect, I don't think that the problem is the 'vague > wording of the rules.' On the contrary, I think "the rules", as a > whole, are pretty clear. > > FCC rules state that you can not operate outside of your own privileges > unless there is a control operator present. > > If a control operator is present, by definition someone who is in > physical control of the operation of the station, in a situation like > this, it stands to reason that there is more than one operator present. > > Therefore this should have, by definition, been entered as a multi- > operator effort. > > Fairly cut and dried. > > Yes, it is unfortunate, and Yuri has my sympathies. Yes, one can argue > that the DQ ruling, based on what little information we know, has the > appearance of being very strict and draconian. (And in all fairness, we > do NOT know the entire story, only what Yuri has told us, so there may > be more to the > story) > > But I just don't see a need to reword the rules to cover an unusual & > near unique situation that actually IS covered. > > 73, ron w3wn > > -----Original Message----- > From: email@example.com > [mailto:firstname.lastname@example.com] On Behalf Of Dick Green WC1M > Sent: Saturday, June 23, 2012 12:01 PM > To: 'W0MU Mike Fatchett'; firstname.lastname@example.com > Subject: Re: [CQ-Contest] KP2MM Disqualified in ARRL CW 2012 > > I think it's somewhat silly and a bit of a stretch to call the presence > of the control operator "assistance". That person isn't doing anything, > other than lending the contest operator use of his/her license. That > person is also lending the contest operator use of his/her station, QTH, > food, bathroom, etc. Why is that different? In no case is the control > operator actually doing anything that affects the competition. He/she is > not operating, providing spots, fixing broken equipment, etc. (I'm sure > the prohibition against that last item has been violated countless > times.) > > Oh, you say operating in the Extra Class sub-band is a competitive > advantage? Yeah, so is a 4-stack on 20m. But the latter can be "loaned" > to the contest operator, while the former cannot. > > That said, the "angels dancing on the head of pin" interpretation being > used is that the control operator must be physically present by FCC > rule. That's true. So, since another body has to be in the room, it's > automatically multi-op. While I disagree with that strict > interpretation, and think the impact on the competition is the better > way to look at this, I believe HQ is within its rights to interpret the > rule as it has. > > However, I do not think the log should be DQed. It should be > reclassified as multi-op. The op didn't try to cheat. He made an > innocent mistake. > > The real issue here is the vague wording of the rules. If the action is > against the rules, and will result in DQ or reclassification, then the > rules should explicitly say so. > > 73, Dick WC1M > > > -----Original Message----- > > From: W0MU Mike Fatchett [mailto:firstname.lastname@example.com] > > Sent: Saturday, June 23, 2012 1:40 AM > > To: firstname.lastname@example.com > > Subject: Re: [CQ-Contest] KP2MM Disqualified in ARRL CW 2012 > > > > I think Yuri understands. I had a couple of off list emails with him. > > > > He is going to shoot for his Extra so this is not an issue again. > > > > I can understand his pain. He put in the effort and he is probably > > not the 1st nor the last that will do this. > > > > If Herb was there the entire contest or the periods where Yuri was > > operating outside his class, we will never know this then why not make > > it a Multi OP log. > > > > Yuri sometimes the best lessons are the hardest ones. > > > > Get that extra and get back in the contest. Don't let this discourage > > you! > > > > 73 > > > > Mike W0MU > > > _______________________________________________ > CQ-Contest mailing list > email@example.com > http://lists.contesting.com/mailman/listinfo/cq-contest > _______________________________________________ CQ-Contest mailing list firstname.lastname@example.com http://lists.contesting.com/mailman/listinfo/cq-contest
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