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Carbon Steel: ASTM A109 Temper 4Home | Directory | Career | News | Industrial
Glossary » Materials » Alloys » Steel » Carbon Steel » ASTM A109 Temper 4
ASTM A109 Temper 4 is categorized as Carbon Steel. It is composed of (in weight percentage) 0.15% Carbon (C), 0.60% Manganese (Mn), 0.035% Phosphorus (P), 0.04% Sulfur (S), 0.90%(max) Silicon (Si), 0.8%(max) Chromium (Cr), 0.20min% Copper (Cu), 0.05min% Zirconium (Zr), 0.1%(max) Titanium (Ti), 0.0025%(max) Boron (B), 0.005-0.06% Niobium (Nb), and the base metal Iron (Fe).
Steel is the common name for a large family of iron alloys. Steels can either be cast directly to shape, or into ingots which are reheated and hot worked into a wrought shape by forging, extrusion, rolling, or other processes. Wrought steels are the most common engineering material used, and come in a variety of forms with different finishes and properties. Carbon steels alloying elements do not exceed these limits: 1% carbon, 0.6% copper, 1.65% manganese, 0.4% phosphorus, 0.6% silicon, and 0.05% sulfur.
The typical elastic modulus of carbon steels at room temperature (25°C) ranges from 190 to 210 GPa. The typical density of carbon steels is about 7.85 g/cm3. The typical tensile strength varies between 276 and 1882 MPa. The wide range of ultimate tensile strength is largely due to different heat treatment conditions.
Additional Information
Other common Carbon steels include AISI 1005, AISI 1006, AISI 1008, AISI 1010, AISI 1011, AISI 1012, AISI 1013, AISI 1015, AISI 1016, AISI 1017, AISI 1018, AISI 1019, AISI 1020, AISI 1021, AISI 1022, AISI 1023, AISI 1025, AISI 1026, AISI 1029, AISI 1030, AISI 1035, AISI 1037, AISI 1038, AISI 1038H, AISI 1039, AISI 1040, AISI 1042, AISI 1043, AISI 1044, AISI 1045, AISI 1045H, AISI 1046, AISI 1049, AISI 1050, AISI 1052, AISI 1053, AISI 1055, AISI 1059, AISI 1060, AISI 1064, AISI 1065, AISI 1069, AISI 1070, AISI 1074, AISI 1075, AISI 1078, AISI 1080, AISI 1084, AISI 1085, AISI 1086, AISI 1090, AISI 1095, AISI 1110, AISI 1117, AISI 1118, AISI 1137, AISI 1139, AISI 1140, AISI 1141, AISI 1144, AISI 1145, AISI 1146, AISI 1151, AISI 1211, AISI 1212, AISI 1213, AISI 1215, AISI 12L14, AISI 1513, AISI 1518, AISI 1522, AISI 1522H, AISI 1524, AISI 1524H, AISI 1525, AISI 1526, AISI 1526H, AISI 1527, AISI 1536, AISI 1541, AISI 1541H, AISI 1547, AISI 1548, AISI 1551, AISI 1552, AISI 1561, AISI 1566, AISI 1572, AISI 15B21H, AISI 15B35H, AISI 15B37H, AISI 15B41H, AISI 15B48H, AISI 15B62H, AISI M1008, AISI M1010, AISI M1012, AISI M1015, AISI M1017, AISI M1020, AISI M1023, AISI M1025, AISI M1031, AISI M1044, ASTM A109 Temper 1, ASTM A109 Temper 2, ASTM A109 Temper 3, ASTM A109 Temper 5, ASTM A284 Grade A, ASTM A284 Grade B, ASTM A284 Grade C, ASTM A284 Grade D, ASTM A285 Grade A, ASTM A285 Grade B, ASTM A285 Grade C, ASTM A288, ASTM A366, ASTM A414 Grade A, ASTM A414 Grade B, ASTM A414 Grade C, ASTM A414 Grade D, ASTM A414 Grade E, ASTM A414 Grade F, ASTM A414 Grade G, ASTM A442 Grade 55, ASTM A442 Grade 60, ASTM A455 Type I, ASTM A455 Type II, ASTM A515 Grade 55, ASTM A515 Grade 60, ASTM A515 Grade 65, ASTM A515 Grade 70, ASTM A516 Grade 55, ASTM A516 Grade 60, ASTM A516 Grade 65, ASTM A516 Grade 70, ASTM A537, ASTM A569, ASTM A570 Grade 30, ASTM A570 Grade 33, ASTM A570 Grade 36, ASTM A570 Grade 40, ASTM A570 Grade 45, ASTM A570 Grade 50, ASTM A573 Grade 58, ASTM A573 Grade 65, ASTM A573 Grade 70, ASTM A606, ASTM A607 Grade 45, ASTM A607 Grade 50, ASTM A607 Grade 55, ASTM A607 Grade 60, ASTM A607 Grade 65, ASTM A607 Grade 70, ASTM A611 Grade A, ASTM A611 Grade B, ASTM A611 Grade C, ASTM A611 Grade E, ASTM A612, ASTM A619, ASTM A620, ASTM A621, ASTM A622, ASTM A662 Grade A, ASTM A662 Grade B, ASTM A678 Grade A, ASTM A678 Grade B, ASTM A678 Grade C, ASTM A715, and ASTM A724 Grade A.
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The October 2014 Corn Hill Gazette is now available to download.
The October 2014 Corn Hill Gazette is now available to download.
CHNA Historian Bonny Mayer and neighbors from the Corn Hill Neighborhood Association will provide one-hour round-trip walking tours from Corn Hill Landing to the Broad Street Aqueduct and back to Corn Hill Landing on Saturday and Sunday October 4 and 5, 2013.
The walking tours will begin at 10 a.m. on Saturday (11 a.m. Sunday) and leave from the Mary Jemison dock at Corn Hill Landing, 290 Exchange Blvd, every hour until 3 p.m. on Saturday and Sunday.
Also, a tour of the Broad Street Aqueduct will be open to the public from noon to 3 p.m. on Saturday October 5.
The work on S. Fitzhugh is scheduled to be done by the end of October. When it's complete, there will be 6 metered parking spaces situated there.
Click this link to view the plans provided by Transportation Control Board.
Cornhill.org now has a direct link to the Flying Squirrel Community Space website from the Useful Links page on this website.
The Flying Squirrel Community Space exists to provide a welcoming space that will cultivate and sustain long lasting relationships between artists, activists, and community members in Rochester so that we may work together to create positive social change. More about us. Check out the current Flying Squirrel Member Groups that use the space. If the Flying Squirrel's mission interests you, then you are encouraged to view their website for upcoming events.
Flying Squirrel Website – http://flyingsquirrel.rocus.org/
Flying Squirrel Facebook Page – https://www.facebook.com/flyingsquirrel.communityspace
285 Clarissa St.
Rochester NY, 14608
To develop a plan to create parallel bicycle-friendly streets along key arterial corridors characterized by high automobile traffic volumes, high parking demand, and/or constrained rights of way which make standard on-street bicycle lanes difficult to achieve.
The City of Rochester completed the Rochester Bicycle Master Plan (BMP) in January 2011 to guide the City's investment in on-street bicycle facilities and bicycle-supportive services. A major product of the BMP is a prioritized list of arterial and collector streets for on-street bicycle improvements. Many of the City's arterial and collector streets possess high traffic volumes, high parking demand, and/or constrained rights-of-way which make bicycle enhancements such as on-street bicycle lanes or shared-use lanes difficult to achieve. In such cases, the development of a parallel or "one-off" network of low-traffic, bike-friendly streets should be considered.
Bicycle boulevards, also known as "neighborhood greenways," are a series of inter-connected streets which have been modified to provide enhanced accommodation as through streets for bicyclists while discouraging through automobile traffic. The BMP identified bicycle boulevards as an increasingly-popular technique nationwide and recommended that the City pursue their implementation locally. The City has engaged Alta Planning + Design to develop the Rochester Bicycle Boulevard Plan, which will make recommendations for creating a network of bicycle boulevards in the City of Rochester with a focus on alternatives to streets for which on-street bicycle facilities are challenging and/or to provide connections between key destinations. Identification of adjacent, low-volume neighborhood streets as bicycle boulevards will greatly enhance Bicycle Level of Service in these difficult corridors and in so doing, will greatly strengthen bike friendliness in our city.
Study Coordination (September 2013 – June 2014)
Develop Prioritization Criteria & Select Plan Corridors (September – December 2013)
Public Meeting #1 (February 11, 2014)
Identify Potential Bicycle Boulevards & Analyze Existing Conditions (January –March 2014)
Make Recommendations & Produce Draft Rochester Bicycle Boulevards Plan (March – September 2014)
Public Meeting #2 (October 2014)
Produce Final Rochester Bicycle Boulevards Plan (December 2014)
Public Involvement
Two "pop-up" public meetings will be held concurrently to provide an overview of the proposed bicycle boulevard route network and the preliminary prioritization of those routes.
Both pop-up meetings will be located along pilot one-mile Bicycle Boulevard routes that the City will install using temporary signage, markings, and traffic calming features. The West Pilot Route will run from Ravenwood Ave. at Genesee Park Blvd. to Frost Ave. at Rugby Ave.; the East Pilot Route will run from the Genesee Riverway Trail at Averill Ave. to Meigs St. at Monroe Ave. The pilot routes will be in place for approximately two weeks, giving residents and visitors ample opportunity to try them out.
Click Here for more information.
It's that time of the year again that the Flower City Work Camp starts accepting referrals for free home repairs. The Flower City Work Camp is a ministry of area churches that will be in its 30th year. During the Spring recess of 2015 they will reach out, with over 700 youth and over 300 adult supervisors, to designated City locations and work for three days doing home repairs at no cost to the home owner. They are now accepting referrals due by November 1st. To best qualify the applicants must be of low to moderate income and have a owner occupied single family home. FCWC is looking for a variety of inside and outside work to keep them busy for three days. Typical work that is done includes: painting, minor plumbing and electrical repair, drywall, windows, doors, flooring, porch and garage repairs. They cannot do roof or gutter repairs that are over one story. Referrals will start to be evaluated in November/December, homes chosen in January/February and work to be completed during Spring Recess of April 2015.
Just give me a name, address and phone number and I will contact the person, explain the program and submit the referral. This program really helps some of our most neediest people and really gets results. Last year they worked on over 60 homes. Please help pass the word. If you would like more information please reply to this e-mail or contact me at Councilwoman Carla Palumbo's office at 428-5982.
Chuck Stechna
Legislative Aide to Carla M. Palumbo
Rochester City Council
North West District Councilwoman
City Hall Room 303A-3
30 Church Street
Rochester, New York 14614
(585) 428-5982 Office
(585) 428-6347 Fax
The CHNA Security Committee is looking for more Corn Hill neighbors who are willing to be trained to walk PAC-TAC patrols in our neighborhood.
Crime Prevention Officer Vincent Agnone will join with Security Chair Marty Petrella for training sessions on Tuesday, October 14 and Tuesday November, 11 at 6:30 p.m. in the lower level conference room at 133 S. Fitzhugh Street prior to the neighborhood's General Meetings.
Please RSVP to firstname.lastname@example.com, sign up to train and help keep our neighborhood safe.
The walk to end Alzheimer's will return to Corn Hill for the 4th year on Saturday October 11th from 9 AM to 1 PM.
85 Adams Street, Rochester, NY
For more information click on Event Details ›
The September 2014 Corn Hill Gazette is now available to download.
The inaugural MS "ROC the Ride Bike Tour" will include Corn Hill. CHNA Historian Bonny Mayer will greet participating bicyclists at noon in the gazebo at Lunsford Circle Park on Sunday, September 7 to offer neighborhood history and information regarding our Historic Wellness Trail.
ROC the Ride's start and finish location is in Genesee Vally Park at 133 Elmwood Avenue. It is anew, one-day, Rochester-centric ride that brings you by some of Rochester's points of interest and gives a fresh look at some interesting spots around the city. The 15 mile Tour of ROC ride for Bike MS is totally different from the usual bicycling event. You won't be continually riding – it is a tour of the city. This is a ride for people who don't usually do charity rides. You don't have to be an athlete, wear bike clothing, know how to follow a bike route, have a fancy bike, or have a lot of experience. This is really an opportunity for people to take an organized, sponsored, guided, informative tour of their community, to make a modest donation to a worthwhile charity by doing so, and to come away with the impression: "WOW! I learned that …, I never knew that. And, you know what, I did it on my BIKE and it was FUN!"
There are 8 stops on the tour; there will be a historian at each stop to give information — it's amazing how many connections there are between these locations and bicycles. It sounds really fun. We are estimating the ride will be about 3 hours total. And there will be separate groups leaving the park. Each group will have 10 riders, a ride leader and a ride "sweep" (total of 12 people in each group). The sweep makes sure no one is left behind. There are only a few miles between each stop. The historian will not begin until all riders in the group have arrived. The stops are:
In addition to the Tour of ROC, several other routes for all cycling abilities allow you to choose the ride that challenges or inspires you! Options include a 30-mile cruise along the Rochester waterway paths; a 45-mile trail ride through the Genesee River Trail, Lehigh Valley Trail, Auburn Trail and more; and a metric century and century ride along the beautiful lakeshore. Each route is fully supported so whichever ROC the Ride route you choose, you will be sure to have a wonderful ride while helping your friends and neighbors who live with the challenges of MS. Register today!
The ride is fully supported with catered rest stops, bike mechanics, full meals and support vehicles. Invite your family and friends to cheer as you cross the finish line, and then enjoy a wonderful afternoon celebration with great food, music, adult beverages, messages, and fun! Bike MS will take you further than you've ever gone before. It's not the miles that matter — it's the unforgettable journey. Join us for an event that's more than a ride — it's anticipation, camaraderie, personal accomplishment, and the knowledge that you're changing lives…making every mile that much sweeter.
Registration Fee: $25,
Fundraising Minimum: $250,
Route Options: 100 miles, 62 miles, 45 miles, 30 miles, and 15 mile tour,
Age Minimum: 12,
Event Manager: Katie Wells
Email: firstname.lastname@example.com,
Phone: 518-464-0850,
Tour of ROC organizer: Scott Wagner
Email: firstname.lastname@example.com,
Phone: 585-880-7643
133 South Fitzhugh Street
Rochester, NY 14608
Office Public Hours
Monday: 2:30-6:30
Wednesday: 2:30-6:30
Other times by appointment.
Contact the CHNA

subset: commoncrawl


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|Amazon Price
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Vinyl, Limited Edition, February 25, 2014
"Please retry"
Audio, Cassette, Live, October 17, 1990
"Please retry"
Product Details
Would you like to update product info or give feedback on images?.
|Disc: 1
|1. Workin' for MCA
|2. I Ain't the One
|3. Saturday Night Special
|4. Searching
|5. Travelin' Man
|6. Simple Man
|7. Whiskey Rock-A-Roller
|8. The Needle and the Spoon
|9. Gimme Back My Bullets
|10. Tuesday's Gone
See all 11 tracks on this disc
|Disc: 2
|1. Gimme Three Steps
|2. Call Me the Breeze
|3. T for Texas (Blue Yodel No. 1)
|4. Sweet Home Alabama
|5. Crossroads
|6. Free Bird
A lot of you kids are too young to remember what Audio is supposed to sound like but under most conditions, this album makes me remember the big boy amps made by Marrantz, Sansui... Read morePublished 4 months ago by Joe M. Williams
Great CD at a great price. I had tried to find this original version previously but was unable to do so, except a few at quite high prices.Published 8 months ago by James R Libbey
Very nice album and songs however the past editions had two more songs. Thought that this was the same albumPublished 9 months ago by Paulo Tarnapolsky
there was a 1 disc release in 1990 that shows cover as "original recording" when it IS NOT!
it omits 2 songs to fit on 1 disc like Little Feat Waiting For Columbus, Amazon... Read more
I am not happy it is missing two or three songs from
the LP, and it did not say this before it was bought.
i love this album but look at the song list it misleads u listing simple man and gimme back my bullits and to mention t for texas but when u get it there not included oh fine print... Read morePublished on May 3, 2012 by SRV FAN
Simply stated, if you do not own this, you are not a fan of good music. Yes the sound quality may not be on par with today's standards. Read morePublished on May 12, 2006 by PickJazz
ONE MORE FROM THE ROAD, along with SECOND HELPING, STREET SURVIVORS, NUTHIN' FANCY, GIMME BACK MY BULLETS, PRONOUNCED LEHN-NERD SKIN-NERD, the FREEBIRD movie soundtrack, and... Read morePublished on May 20, 2005 by The Footpath Cowboy


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Category:Haumea (dwarf planet)
From Wikipedia, the free encyclopedia
|Wikimedia Commons has media related to Haumea.
The main article for this category is Haumea (dwarf planet).
Haumea is a dwarf planet in the Kuiper belt, and named after the Hawaiian goddess of childbirth – see Haumea (mythology).
This category has only the following subcategory.
- ► Moons of Haumea (3 P)
Pages in category "Haumea (dwarf planet)"
The following 5 pages are in this category, out of 5 total. This list may not reflect recent changes (learn more).


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Swiss Co. Accused of Stiffing U.S. $750 Million for Food to Troops in AfghanistanSubmitted by go213mph on Sun, 05/27/2012 - 09:23
NOTE: What burns me up about this story is why in the hell was the DoD buying food for our troops from a company in Switzerland? The US economy could use the "business". Of course I know the reason...war is a racket. When it was decided that a war needed to be fought to enrich war profiteers I guess they had to include the swiss multinational corporations in the spoils of war too. Even though I don't beleive the swiss committed a single solider.
"Seven years after it was hired to provide food to troops in Afghanistan, a Swiss company has been accused of overcharging the Department of Defense about three-quarters of a billion dollars.
Supreme Foodservice AG of Switzerland has been paid $5.5 billion since 2005 to deliver water and food, including fresh fruit and vegetables, to U.S. forces at about 246 sites in Afghanistan"
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Speaking of RACKET?
Has anyone watched War Inc. ???
"The only thing we die with is our own personal integrity!" LRH
Vail, CO
Freedom and Liberty for Eagle County, Colorado. www.flecc.org
Milo Minderbinder
And that's the catch.
Free includes debt-free!
Swiss Co. - A (Not) Funny Coincidence -
Reading your comment, I googled Supreme to see what was so special about it: seems they've perfected the art of delivering food to "troubled areas." I hastily penned the following comment, "If you look up their niche, and given that Americans are both innovative and entrepreneurial, no doubt it's been our GOV'T REGULATIONS that precluded a U.S. company being able to provide the same. http://www.supreme-group.net/index.php?pageid=425" But, I decided to first read more about it. Am I smart! It was founded by AN AMERICAN, a former U.S. Army food service soldier. http://www.supreme-group.net/index.php?pageid=451 I also noticed that a "Supreme U.S.A." was founded in 2009. I don't have all day here, but I threw it into the googlesphere, and because of the words I used, an article happened to come up: the SUPREME Court threw out a case brought by opponents of a similar FOODSERVICE company start-up. The problem? Not state or federal gov't regulations, but local zoning plans - in line with an INT'L agenda. Buda, TX is a "Greentown" = ICLEI = Agenda 21. http://haysfreepress.com/2010/06/11/supreme-court-throws-out...
When we try to pick out anything by itself, we find it hitched to everything else in the Universe.
~ John Muir
Good Finds Mdef
Well, "good" is probably the wrong choice of words but thanks for the info.
"It's a wonder we can even feed ourselves."
Thank you for the enlightening post. It burns me up, too!!! Between the corporate ties to government and all the lobbying that goes on and behind-closed-doors deals, including the priorities of the military-industrial complex; along with all the federal regulations by these myriad non-elected "czars" who, against the Constitution, have become de facto legislators; along with the country's signing onto U.N. policies like Agenda 21, with its map already drawn for allowable land-use in America (and Obama's have already usurped control over farmland); and also U.N. policies like Codex Alimentarius that, in the name of international trade/re-distribution of resources thwarts our food freedom and has citizens who wish to distribute non-irradiated, organic "raw foods" thrown into jail; along with what would seem to be the deliberate dumbing down of America via our failing education system... I couldn't help but think of the last line of this Dylan song, IDIOT WIND. http://www.videohippy.com/video/158185/Idiot-Wind--Bob-Dylan
When we try to pick out anything by itself, we find it hitched to everything else in the Universe.
~ John Muir


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This is a popular cruise ship departure port, so often the hotels fill up just before sailing time. In any case, this hotel should be a "last resort" if needed. The rooms are Spartan, roomy enough for a night or 2, with Spartan bathrooms and virtually no amenities. There is decent WIFI in the lounge only. The shower in the... More
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- TripAdvisor is proud to partner with Expedia, Travelocity, Booking.com, Hotels.com, Priceline, Despegar.com, Odigeo, TripOnline SA and Tingo so you can book your Hotel Mediterraneo reservations with confidence. We help millions of travelers each month to find the perfect hotel for both vacation and business trips, always with the best discounts and special offers.
- Also Known As:
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Celeb designer teams up with Sarasota art studio
Date: August 4, 2010
by: Heidi Kurpiela | A&E Editor
Ed Marin has only lived in Sarasota one year and already he's bringing A-list, interior-design stars to town.
Marin, owner of Soicher Marin, a high-end design studio that specializes in outfitting luxury spas and hotels with boutique art, launched into a new project last month with celebrity interior designer Thom Filicia.
Filicia, the interior-design expert on the Bravo reality show, "Queer Eye," now hosts two home-makeover shows on the Style Network — "Dress My Nest" and "Tacky House" — in addition to designing his own furniture line, which is where Marin comes into play.
Filicia had been looking for an art-design company to produce pieces for his new Thom Filicia Home Collection. A friend in California suggested he call Marin, whose company was a Los Angeles mainstay for 55 years, before Marin relocated it in November to North Sarasota.
"We hit it off right away," says Marin, whose wife runs Yoga SRQ in Burns Court. "He's not pretentious in any way. Everything about the guy is genuine. What you see is what you get, and having worked with him, I can tell you, he's got great ideas."
Marin and Filicia met in early July over a whirlwind three days. On the last night of Filicia's stay, they pulled an all-nighter inside Marin's studio, brainstorming ideas, matching mattes and frames, running image ideas back and forth to Marin's design team.
"His stuff is accessible," Marin says. "You don't have to be a genius to figure it out. It takes me back to being a kid; the nostalgia of America, of a time that was perhaps simpler."
Filicia was drawn to minimalist designs: a handful of leaves, an out-of-focus beach scene, airplanes, and other smatterings of Americana.
After working nearly 24 hours straight, the men decided to break. They met later that evening for dinner at Mediterraneo in downtown Sarasota, where Filicia shook hands with the cooks, the busboys and the wait staff.
"The purpose of the meeting was to process and digest what had been a very intensive e-mail dialogue into a plan of attack," Marin says. "It was extremely productive, but by the end of the last day, we were toast."
The Soicher Marin-Filicia collaboration has been a year in the making. Marin and Filicia have exchanged dozens of e-mails outlining how the artwork should look, its price point and the importance of dovetailing it with the rest of Filicia's line.
Marin's company has until September to finalize the designs. In October, they'll unveil the collection with Filicia at High Point Market, a furniture trade show held in High Point, N.C., that attracts more than 100,000 furniture exhibitors and buyers from around the world.
The pieces will eventually be sold with the rest of Filicia's collection at Robb & Stucky.
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- Nov
25 Cine Selby
5:30 pm
- Nov
28 The Jazz Club of Sarasota Presents "Jazz at Two" with Alex and Judi Glover
2:00 pm - 4:00 pm
- Nov
28 WSLR Hand-Cranked Festival: Day 1
7:00 pm
- Nov
29 WSLR Hand-Cranked Festival: Day 2
4:00 pm
Cops Corner: Sarasota
Enjoy this week's edition of Cops Corner.
World on a string
The exchange of goods and chatter are the usual soundtrack for the Saturday morning Downtown Farmers Market on Orange Avenue.
Read all about it
Bookstores across the state celebrated Florida Bookstore Day Saturday. Bookstore1 held an all-ages literary party and read-a-thon for customers and members of the community.


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Yet it is Rarely Used according to the metagame.
Sceptile's the only Hoenn starter I like.
I love my Kingdra.
I need to get a good Kingdra.
Kingdra is indeed great, but the current metagame isn't very nice to it and sent it to the low tiers because of its "jack of all stats" status. Same goes to Sceptile because of his "fragile speedster" status.
I ought to get a Kingdra sometime.
I've had a Kingdra for a loooooooong time.
There are many Pokemon I have never used. Mostly just because there are so many now.
There are so many sets to choose from for your Kingdra.
Maybe I can use it in B2/W2 if its available.
Woah, this thread just passed page 1000!
I never had a Kingdra mostly because you have to trade it with an item. I hate pokemon you trade with items to evolve.
I don't always resist, but when I do, it's with The Resistance. Stay resistant, my friends.
Originally Posted by Iron Defense
I hate it too. :< At least they let us catch wild Milotic now, but...
Bishounen Pride! • Claims: Ayane, Xingcai • Avatar/Signature
Should be studying for math test. Math too boring.


text:
Home » Opinion » Columns
Pat Buchanan: The new Pope and the decline of the West
By 1938, Belloc concluded Christian Europe was done:
"The bad work begun at the Reformation is bearing its final fruit in the dissolution of our ancient doctrines - the very structure of society is dissolving." He was right. Europe is the dying continent.
And looking back at the history of the Old Continent, we see the truth of G.K. Chesterton's insight: When men cease to believe in God, they do not then believe in nothing, they will believe in anything.
Consider the idols to which European Man has burnt incense since losing his faith: Darwinism, Marxism, Bolshevism, fascism, Nazism, now globalism - the idea of a secular paradise where mankind's needs are met by the state and people spend their lives consuming cultural and material goods until the time comes for the painless exit.
Last Wednesday, even as Europe has said goodbye to Rome, Rome began to say goodbye to Europe, where the fastest growing faith is manifest in the mosques rising from Moscow to Madrid.
The College of Cardinals, for the first time ever, chose a Pope from the New World: Cardinal Jorge Bergoglio of Argentina.
To be exact, Pope Francis is not of the indigenous peoples of the New World. His father was an immigrant from Italy who came to Argentina before he was born. Yet, though by blood an Italian, Pope Francis, heart and soul, does not belong to Europe.
The reaction of our secular media to the election of this first Jesuit Pope, who lives his "preferential option for the poor," was easily predictable.
On redistribution - "Is he a conservative, or a Great Society liberal who will push the 'social gospel'?" - the new Pope passes with honors. He has a simple apartment, rides the bus and lives among the Buenos Aires poor.
But on the "social issues" - "Is Pope Francis a progressive who will move the Church to a more 'tolerant' view of abortion and same-sex marriage?" - the disappointment of the media elite was evident.
Pope Francis adheres to orthodox Catholic teaching that abortion is the killing of an unborn child entailing automatic excommunication for all involved. He has denounced same-sex marriage and regards homosexual adoptions as a crime against children.
That the media showed visible disappointment at learning this makes one wonder if they know anything at all about the Catholic Church.
To be Catholic is to be orthodox.
Indeed, let us presume the impossible - that the Church should suddenly allow the ordination of woman, and decree that abortions in the first month of pregnancy are now licit, and that homosexual unions, if for life, will henceforth be recognized and blessed.
This would require the Church to admit that for 2,000 years it had been in error on matters of faith and morals, and hence is not infallible. But if the Church could have been so wrong for so long, while the world was right, and many had suffered for centuries because the Church erred, what argument would be left for remaining Catholic?
If the Church were to admit it had been wrong since the time of Christ about how men must live their lives to attain eternal life, why should Catholics obey the commandments of such a fallible and erring Church? Why not follow our separated brethren of the Protestant faiths, and choose what doctrines we wish to believe and what commandments we wish to obey?
And how have those churches fared that have accommodated themselves to the world?
Of the Christian denominations, the closest to Catholicism has been the Anglican or Episcopal Church. For a time, Anglicans were not regarded as heretics. For though they had rejected the primacy of Rome, they had not rejected the truths fundamental to Catholicism. They had been seen in the time of Henry VIII as schismatics.
But lately the Episcopal Church has been in the vanguard of all Christian churches in ordaining women priests and consecrating women and homosexuals as bishops.
Result? No church has suffered greater losses, as Catholicism has benefited from a steady stream of defecting Anglican clergy.
What the secular media reaction to Pope Francis reveals is that traditional Catholicism is today almost as deeply alien to our present-day West as it was in Roman times, only the West chooses to ignore Catholicism, where Rome feared and persecuted it.
As of this writing President Obama was reportedly to send to today's official installation of the Holy Father to represent America our ranking Catholic officeholders, Vice President Joe Biden, along with former Speaker Nancy Pelosi.
One wonders what His Holiness will be thinking as he greets these ornaments of American Catholicism, both of whom regard Roe v. Wade, which has resulted in 53 million abortion deaths, as a milestone of progress for women's rights and homosexual marriage as the civil rights cause of the 21st century.
Pat Buchanan is a former Republican and Reform Party candidate for President, an adviser to two Presidents, a syndicated columnist based in Washington, D.C., and the author of "Suicide of a Superpower: Will America Survive to 2025?"
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Everybody's, including kids', texting and talking options continue to multiply like rabbits. First there was texting from your mobile carrier, then phone-based texting like Apple's iMessage and voice via computers (later adding in phones) as with Google Voice and Skype. Along came texting apps too, such as the simple and spare WhatsApp now owned by […]
Tag Archives | texting
Texting options multiplying like rabbits (even more now)
How Yik Yak is different from other social media
Based on news reports and conversations with educators about troubles with Yik Yak, the location-based anonymous texting app, at schools in many parts of the US, three scenarios leap to mind…. Remember that kid at school (or maybe that frat house) who felt the need or the pressure to set off the fire alarm to […]
Teens on social media's impact on relationships: Survey
Today a guest post that shows how much we need to understand the relationship between social media and social life – in other words, get more granular in our understanding of how social media affect the relationships in our lives (our lives as a whole, not just the online parts)…. Guest post by Kris Gowen […]
With this teen texting study, only methodology was news
The methodology for a just-released study about teens and texting was new, but the findings don't seem to break much new ground – unless the news media had picked up on what the researchers didn't highlight. More on the reporting in a minute; first the study, published by researchers at University of Texas, Dallas, in […]
The One Good Thing project
The vast majority of things that happen online are positive. Let's tell that story - together. Learn more
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"Breaking Bad" Superstar Bryan Cranston Supports New Mexico Unites for Marriage
January 20, 2014 by Maureen McCarty, HRC Associate Director of Digital Media
Bryan Cranston, known for his award-winning role as Walter White on Breaking Bad, recently added his voice to the growing majority who affirm same-sex couples right to marry in the Land of Enchantment.
"I support the work of New Mexico Unites for Marriage to ensure that all couples have the privilege of committing legally and publicly to the institution of marriage," said Cranston in a statement.
Cranston is no stranger to New Mexico or to LGBT advocacy. The megahit television series Breaking Bad was set and produced in Albuquerque. And in 2012, he and his family publicly spoke out in support of marriage equality nationwide, as part of HRC's Americans for Marriage Equality campaign.
HRC is proud to be a founding member of New Mexico United for Marriage, a broad and diverse coalition of local and national organizations, businesses and civic leaders, communities of faith and everyday New Mexicans working to secure and protect marriage equality in the state.
In December, the New Mexico Supreme Court issued the historic ruling to extend marriage equality statewide. However, conservative legislators continue to fight to deny loving, committed couples the right to marry.
A hallmark of the LGBT rights movement is the fight to have our relationships recognized as equal under the law. Full marriage equality is the true measure of our success in this area. To learn more about the marriage equality landscape and how you can get involved, visit HRC's Marriage Center.
And to help Cranston and New Mexico Unites for Marriage defend marriage equality, please contact HRC's New Mexico field organizers Leigh and Marshall.
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Please join us for the first Houston appearance of Mr.Kitty!
with special guests: MKF Kunst Fasción & Provision. Alongside Be.Witched Dj's playing tunes all night!
A LIVE concert & dance-party happening the night before Thanksgiving! So most of us should be clear of work and school!
Mr.Kitty is a fragile hologram projected by illuminated crystals. With brittle synths, pulsating rhythms, and soaring vocals, he will be the light to guide you through the darkness.
MKF Kunst Fascion
(Houston, Tx.)
(Houston, Tx.)
w/ Be.Witched Wednesdays Dj's DJ Damon Allen & Franki Franki.
Doors: 9pm / Show starts at 10pm
Presale tickets are cheaper starting with a limited amount of 5$ tickets: Order tickets via Eventbrite:
Order Tickets
***At door tickets will be 10$ for 21 up / 12$ under 21***
Event is 18 and up!
Open EARLY @ 7:00 w/ all male Burlesque!
Classic Numbers BLACK CELEBRATION 5
immediately following @ 9:45
W/DJs: Mina and vvJames
Spinning Goth / EBM / Industrial
Cover: $5 for over 21/ $10 for under 21
Doors: 9pm
$2 Wells & Domestics til' 11pm
$2 Lone Star & PBR All Night
$3 Jager & Fireball All Night
DJ Enigma (CybeRot, HTX) and Vampire James (The Underworld, HTX) EBM/Industrial
$5.00/ 21 and up
$10.00/ 20 and under
Doors at : 9pm
BIG Gift Giveaway including XBOX 1, iPod Nano, iPad Mini, Lifetime Passes, Erotic Cabaret Gift Certificates, T-Shirts, Mix CDs & MORE!
NO COVER til 10:00
DOLLAR DRINKS till 11:00!
with all of your favorite people & all your favorite music!
More Details Tbp ...
"Capricorn Ball"
W/DJs: Mina and vvJames
Guest DJ: Hideous and Roger Wilson (★ New Neon ★ NYC)
Spinning Goth / EBM / Industrial / Electro
*Performance by: EBM Dollz (Austin)
*Vendors Area*
*Upstairs Cappy VIP Area* - B-day Treats &
Drink Specials
Cover: $5 for over 21/ $10 for under 21
Doors: 9pm
$2 Wells & Domestics til' 11pm
$2 Lone Star & PBR All Night
$3 Jager & Fireball All Night
Start the year out right with a 6400 party with all the original music. With Number's BRAND NEW light show, LeRoy Peters and Dj Xcess djing, the party will be going strong all night. Tell all your friends and lets pack the house!!
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Fugitive lip biter arrested in California
Nov 6, 2014
Jonathan E. Benedict
Oct 30, 2014
Darrell W. Cook
Oct 2, 2014
Michael Polchies
Sep 25, 2014
Home » News » Crime
TSA agent killed, 6 wounded in Los Angeles airport shooting
Passengers evacuate after a shooting incident at Los Angeles International Airport on Friday. (REUTERS/Lucy Nicholson)
A spokesman for the Los Angeles County Coroner said it was handling one person who was killed in the shooting, a man approximately 40 years old who was not identified by name. It was not immediately clear if that person was the TSA agent.
Three male victims hurt in the incident were taken to Ronald Reagan UCLA Medical Center, where one was listed in critical condition and two others in fair condition, said Mark Wheeler, a spokesman for the hospital.
'Shooting randomly'
Witness Alex Neumann, meanwhile, told cable network CNN that he was in an area inside the airport past a security checkpoint when he heard loud noises and screaming and saw people running.
President Obama was briefed on the incident and White House officials are in touch with law enforcement officials on the ground, White House spokesman Jay Carney told reporters.
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Jury acquits Mont Vernon driver, who was checking text, in death of former Amherst fire chief
Obama to illegals: Come out of shadows
Offend someone? To jail with you!
Jury acquits Mont Vernon driver, who was checking text, in death of former Amherst fire chief
Plaistow town planner arrested
NH asks feds for waiver on Medicaid
Driver acquitted in Amherst ex-chief's death
- Whom do you think bears the brunt of the blame for the mayhem this weekend in Keene?
- KSC students
- 37%
- KSC administration
- 2%
- Police
- 6%
- Visitors from out of town
- 18%
- A combination of any/all of the above
- 37%
- Total Votes: 2483


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Publications (144)471.72 Total impact
Article: CHARGE-like presentation, craniosynostosis and mild Mowat–Wilson Syndrome diagnosed by recognition of the distinctive facial gestalt in a cohort of 28 new cases[Show abstract] [Hide abstract]
ABSTRACT: Mowat–Wilson syndrome (MWS) is characterized by moderate to severe intellectual disability and distinctive facial features in association with variable structural congenital anomalies/clinical features including congenital heart disease, Hirschsprung disease, hypospadias, agenesis of the corpus callosum, short stature, epilepsy, and microcephaly. Less common clinical features include ocular anomalies, craniosynostosis, mild intellectual disability, and choanal atresia. These cases may be more difficult to diagnose. In this report, we add 28 MWS patients with molecular confirmation of ZEB2 mutation, including seven with an uncommon presenting feature. Among the "unusual" patients, two patients had clinical features of charge syndrome including choanal atresia, coloboma, cardiac defects, genitourinary anomaly (1/2), and severe intellectual disability; two patients had craniosynostosis; and three patients had mild intellectual disability. Sixteen patients have previously-unreported mutations in ZEB2. Genotype-phenotype correlations were suggested in those with mild intellectual disability (two had a novel missense mutation in ZEB2, one with novel splice site mutation). This report increases the number of reported patients with MWS with unusual features, and is the first report of MWS in children previously thought to have CHARGE syndrome. These patients highlight the importance of facial gestalt in the accurate identification of MWS when less common features are present. © 2014 Wiley Periodicals, Inc.American Journal of Medical Genetics Part A 08/2014; · 2.30 Impact Factor
Article: Unusual 4p16.3 deletions suggest an additional chromosome region for the Wolf-Hirschhorn syndrome–associated seizures disorder[Show abstract] [Hide abstract]
ABSTRACT: Objective Seizure disorder is one of the most relevant clinical manifestations in Wolf-Hirschhorn syndrome (WHS) and it acts as independent prognostic factor for the severity of intellectual disability (ID). LETM1, encoding a mitochondrial protein playing a role in K+/H+ exchange and in Ca2+ homeostasis, is currently considered the major candidate gene. However, whether haploinsufficiency limited to LETM1 is enough to cause epilepsy is still unclear. The main purpose of the present research is to define the 4p chromosome regions where genes for seizures reside.Methods Comparison of our three unusual 4p16.3 deletions with 13 literature reports. Array-comparative genomic hybridization (a-CGH). Real-time polymerase chain reaction (RT-PCR) on messanger RNA (mRNA) of LETM1 and CPLX1. Direct sequencing of LETM1.ResultsThree unusual 4p16.3 deletions were detected by array-CGH in absence of a obvious clinical diagnosis of WHS. Two of these, encompassing LETM1, were found in subjects who never had seizures. The deletions were interstitial, spanning 1.1 Mb with preservation of the terminal 1.77 Mb region in one case and 0.84 Mb with preservation of the terminal 1.07 Mb region in the other. The other deletion was terminal, affecting a 0.564 Mb segment, with preservation of LETM1, and it was associated with seizures and learning difficulties. Upon evaluating our patients along with literature reports, we noted that six of eight subjects with terminal 4p deletions preserving LETM1 had seizures, whereas seven of seven with interstitial deletions including LETM1 and preserving the terminal 1 Mb region on 4p did not. An additional chromosome region for seizures is suggested, falling within the terminal 1.5 Mb on 4p, not including LETM1.SignificanceWe consider that haploinsufficiency not limited to LETM1 but including other genes acts as a risk factor for the WHS-associated seizure disorder, according to a comorbidity model of pathogenesis. Additional candidate genes reside in the terminal 1.5 Mb region on 4p, most likely distal to LETM1.A PowerPoint slide summarizing this article is available for download in the Supporting Information section here.Epilepsia 04/2014; · 3.96 Impact Factor
Article: Clinical, neurophysiological and pathological findings of HNPP patients with 17p12 deletion: A single-centre experience[Show abstract] [Hide abstract]
ABSTRACT: Background Classic clinical manifestations of HNPP are characterized by recurrent painless mononeuropathies, but a minority of patients presents with atypical clinical pattern, including CMT-like neuropathy, acute or chronic inflammatory demyelinating neuropathy-like polyneuropathy, carpal tunnel syndrome. Electrophysiological examination plays a central role in the diagnosis of HNPP, disclosing a non-uniform conduction slowing, more pronounced at entrapment sites. Patients and Methods We report clinical, electrophysiological and pathological findings from 73 patients with HNPP, coming from 53 unrelated families, followed in our Institute of Neurology over a 20-year period. Results Typical presentation with recurrent multiple mononeuropathies was observed in 28/64 (44%) patients. In the remaining 36/64 (56%), we observed an atypical clinical presentation, characterized by generalized weakness and cramps; chronic ulnar neuropathy; carpal tunnel syndrome; chronic sensory polyneuropathy; Guillain-Barrè-like presentation; CMT-like presentation. Nine patients were asymptomatic for neuropathic symptoms. Nerve conduction studies showed in all cases a sensori-motor demyelinating polyneuropathy with conduction abnormalities preferentially localized at common entrapment sites. When performed, sural nerve biopsy disclosed focal thickenings of the myelin sheath in all patients. Conclusions About half of patients with HNPP from our cohort showed an atypical clinical presentation. Neurophysiological examination represents the main tool for a proper diagnosis.Journal of the neurological sciences 01/2014; · 2.32 Impact Factor
Article: Interstitial deletion of 3p22.3p22.2 encompassing ARPP21 and CLASP2 is a potential pathogenic factor for a syndromic form of intellectual disability: A co-morbidity model with additional copy number variations in a large family.[Show abstract] [Hide abstract]
ABSTRACT: We describe a family in which four individuals (the mother and three children) presented with an overlapping phenotype of minor physical anomalies and intellectual disability. Four previously unreported copy number variants were found inherited either from the affected mother or from the healthy father, consisting of a 3p22.3p22.2 deletion (2.5 Mb), a 3p24.3 deletion (0.55 Mb), a 6q22.31 duplication (0.74 Mb), all maternally inherited, and an 18q11.2 duplication (0.276 Mb) which was paternally inherited. The deletions on chromosome 3 were both found to segregate with the disease. However, being the 0.55 Mb deleted segment on 3p24.3 devoid of genes, we considered that the 2.5 Mb deletion on 3p22.3p22.2 acts as major pathogenic rearrangement in this condition. Among the transcribed genes residing in this interval, ARPP21 and CLASP2 are proposed as good candidate genes on the basis of their functional properties. A co-morbidity role for the other small rearrangements detected in the affected individuals in association with the 3p22.3p22.2 deletion is also suggested, according to a second-side model of pathogenesis. © 2013 Wiley Periodicals, Inc.American Journal of Medical Genetics Part A 10/2013; · 2.30 Impact Factor
- American Journal of Medical Genetics Part A 10/2013; · 2.30 Impact Factor
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ABSTRACT: To characterize epileptic phenotype, electroencephalography (EEG) features, and epileptic evolution in patients with ring 14 r(14) syndrome. Twenty-two patients with ring chromosome 14 were enrolled in the study. We examined age at onset, seizure semiology and frequency at onset and at follow-up, drug responsiveness/resistance, and interictal/ictal EEG data. The degree of severity of the epileptic phenotype negatively influences child cognitive development. The incidence of epilepsy in patients with r(14) syndrome is virtually 100%, characterized by early onset, polymorphic seizures, and drug-resistant seizures. In addition, we ascertained focal secondarily generalized epilepsy, seizure cluster tendency, frequent status epilepticus, and a rather typical epilepsy evolution. EEG abnormalities consisted of slow background activity with pseudoperiodic bursts of generalized slow waves in the early stage, focal frontotemporal or temporoposterior slow waves with multifocal spikes interposed, and unusual rhythmic fast recruiting posterior spikes followed by secondary generalization. The degree of severity of the epileptic phenotype negatively influences child cognitive development. This study provides a more precise definition of seizure types, natural history, and drug responsiveness of r(14) syndrome, a highly epileptogenic chromosomal condition.Epilepsia 10/2013; · 3.96 Impact Factor
Article: Mutations in the 3' Untranslated Region of FUS causing FUS overexpression are associated with Amyotrophic Lateral Sclerosis.[Show abstract] [Hide abstract]
ABSTRACT: Mutations in the gene encoding FUS have been identified in a subset of patients with sporadic and familial Amyotrophic Lateral Sclerosis (ALS). Variants in the 3' Untranslated Region (3'UTR) of FUS have also been reported in ALS patients, but their pathogenic role has not been assessed.We sequenced the whole 3'UTR of FUS in 420 ALS patients who were negative for mutations in the currently known ALS genes and in 480 ethnically matched controls. We detected four 3'UTR variants (c.*48 G>A, c.*59 G>A, c.*108 C>T and c.*110 G>A) in four sporadic and in one familial ALS patients compared to none in controls (p=0.02).We investigated whether these variants impaired FUS expression in primary fibroblast cultures from three patients harbouring the c.*59 G>A, c.*108 C>T and c.*110 G>A variants, respectively. The pattern of FUS expression was also investigated in fibroblasts from one ALS patient with FUS R521C mutation, in two ALS patients without mutations in the known ALS genes and in four control individuals. By immunostaining and immunoblotting, large amounts of FUS were observed in both the cytoplasm and nuclei of mutant 3'UTR FUS fibroblasts. In FUS R521C mutant fibroblasts we observed a slight increase of FUS in the cytoplasm associated with a remarkable loss of detection in nuclei.Our findings show that mutations in 3'UTR of FUS are overrepresented in ALS patients and result into translation de-regulation of FUS. Overexpression and mislocalization of wild type FUS likely contribute to ALS pathogenesis in these cases.Human Molecular Genetics 07/2013; · 7.69 Impact Factor
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ABSTRACT: The clinical approach to patients with amyotrophic lateral sclerosis (ALS) has been largely modified by the identification of novel genes, the detection of gene mutations in apparently sporadic patients, and the discovery of the strict genetic and clinical relation between ALS and frontotemporal dementia (FTD). As a consequence, clinicians are increasingly facing the dilemma on how to handle genetic counselling and testing both for ALS patients and their relatives. On the basis of existing literature on genetics of ALS and of other late-onset life-threatening disorders, we propose clinical suggestions to enable neurologists to provide optimal clinical and genetic counselling to patients and families. Genetic testing should be offered to ALS patients who have a first-degree or second-degree relative with ALS, FTD or both, and should be discussed with, but not offered to, all other ALS patients, with special emphasis on its major uncertainties. Presently, genetic testing should not be proposed to asymptomatic at-risk subjects, unless they request it or are enrolled in research programmes. Genetic counselling in ALS should take into account the uncertainties about the pathogenicity and penetrance of some genetic mutations; the possible presence of mutations of different genes in the same individual; the poor genotypic/phenotypic correlation in most ALS genes; and the phenotypic pleiotropy of some genes. Though psychological, social and ethical implications of genetic testing are still relatively unexplored in ALS, we recommend multidisciplinary counselling that addresses all relevant issues, including disclosure of tests results to family members and the risk for genetic discrimination.Journal of neurology, neurosurgery, and psychiatry 07/2013; · 4.87 Impact Factor
Article: Mild beckwith-wiedemann and severe long-QT syndrome due to deletion of the imprinting center 2 on chromosome 11p.[Show abstract] [Hide abstract]
ABSTRACT: We report on a young woman admitted to our Cardiology Unit because of an episode of cardiac arrest related to a long-QT syndrome (LQTS). This manifestation was part of a broader phenotype, which was recognized as a mild form of Beckwith-Wiedemann syndrome (BWS). Molecular analysis confirmed the diagnosis of BWS owing to a maternally inherited deletion of the centromeric imprinting center, or ICR2, an extremely rare genetic mechanism in BWS. The deletion interval (198 kb) also included exons 11-16 of the KCNQ1 gene, known to be responsible for LQTS at locus LQT1. No concomitant mutations were found in any other of the known LQT genes. The proposita's mother carries the same deletion in her paternal chromosome and shows manifestations of the Silver-Russell syndrome (SRS). This report describes the smallest BWS-causing ICR2 deletion and provides the first evidence that a paternal deletion of ICR2 leads to a SRS-like phenotype. In addition, our observation strongly suggests that in cases of LQTS due to mutation of the KCNQ1 gene (LQT1), an accurate clinical genetic evaluation should be done in order to program the most appropriate genetic tests.European Journal of Human Genetics advance online publication, 20 March 2013; doi:10.1038/ejhg.2012.280.European journal of human genetics: EJHG 03/2013; · 3.56 Impact Factor
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ABSTRACT: Though clinical picture of ALS is a stereotypical one, resulting from combination of signs secondary to dysfunction of both upper and lower motor neurons, clinical heterogeneity is a consistent feature of the disease. Age of onset, relative mix of upper and lower motor neuron signs, duration of the disease and association with other conditions are major factors contributing to variable clinical phenotypes. Genetically, familial forms of ALS are associated with a large number of pleiotropic genes whose mutations impair different biochemical pathways, resulting in overlapping clinical and pathological phenotypes. Over the last few years contribution of large- and low-effect genes to sporadic ALS is increasingly recognized.Clinical Genetics 02/2013; · 4.25 Impact Factor
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ABSTRACT: Mowat-Wilson syndrome (MWS) is a genetic disease caused by heterozygous mutations or deletions of the ZEB2 gene and is characterized by distinctive facial features, epilepsy, moderate to severe intellectual disability, corpus callosum abnormalities and other congenital malformations. Epilepsy is considered a main manifestation of the syndrome, with a prevalence of about 70-75%. In order to delineate the electroclinical phenotype of epilepsy in MWS, we investigated epilepsy onset and evolution, including seizure types, EEG features, and response to anti-epileptic therapies in 22 patients with genetically confirmed MWS. Onset of seizures occurred at a median age of 14.5 months (range: 1-108 months). The main seizure types were focal and atypical absence seizures. In all patients the first seizure was a focal seizure, often precipitated by fever. The semiology was variable, including hypomotor, versive, or focal clonic manifestations; frequency ranged from daily to sporadic. Focal seizures were more frequent during drowsiness and sleep. In 13 patients, atypical absence seizures appeared later in the course of the disease, usually after the age of 4 years. Epilepsy was usually quite difficult to treat: seizure freedom was achieved in nine out of the 20 treated patients. At epilepsy onset, the EEGs were normal or showed only mild slowing of background activity. During follow-up, irregular, diffuse frontally dominant and occasionally asymmetric spike and waves discharges were seen in most patients. Sleep markedly activated these abnormalities, resulting in continuous or near-to-continuous spike and wave activity during slow wave sleep. Slowing of background activity and poverty of physiological sleep features were seen in most patients. Our data suggest that a distinct electroclinical phenotype, characterized by focal and atypical absence seizures, often preceded by febrile seizures, and age-dependent EEG changes, can be recognized in most patients with MWS. © 2013 Wiley Periodicals, Inc.American Journal of Medical Genetics Part A 01/2013; · 2.30 Impact Factor
Article: A novel compound heterozygous ALS2 mutation in two Italian siblings with juvenile amyotrophic lateral sclerosis.Amyotrophic lateral sclerosis and frontotemporal degeneration. 01/2013;
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ABSTRACT: The gene expression pattern of glioblastoma (GBM) is well documented but the expression profile of brain adjacent to tumor is not yet analysed. This may help to understand the oncogenic pathway of GBM development. We have established the genome-wide expression profiles of samples isolated from GBM tumor mass, white matter adjacent to tumor (apparently free of tumor cells), and white matter controls by using the Affymetrix HG-U133 arrays. Array-CGH (aCGH) was also performed to detect genomic alterations. Among genes dysregulated in peritumoral white matter, 15 were over-expressed, while 42 were down-regulated when compared to white matter controls. A similar expression profile was detected in GBM cells. Growth, proliferation and cell motility/adhesion-associated genes were up-regulated while genes involved in neurogenesis were down-regulated. Furthermore, several tumor suppressor genes along with the (a member of natural killer receptor) were also down-regulated in the peritumoral brain tissue. Several mosaic genomic lesions were detected by aCGH, mostly in tumor samples and several GBM-associated mosaic genomic lesions were also present in the peritumoral brain tissue, with a similar mosaicism pattern. Our data could be explained by a dilution of genes expressed from tumor cells infiltrating the peritumour tissue. Alternatively, these findings could be substained by a relevant amount of "apparently normal" cells presenting a gene profile compatible with a precancerous state or even "quiescent" cancer cells. Otherwise, the recurrent tumor may arise from both infiltrating tumor cells and from an interaction and recruitment of apparently normal cells in the peritumor tissue by infiltrating tumor cells.PLoS ONE 01/2013; 8(3):e57145. · 3.53 Impact Factor
Article: Inherited neuropathies and deafness caused by a PMP22 point mutation: a case report and a review of the literature.Neurological Sciences 12/2012; · 1.41 Impact Factor
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ABSTRACT: The present study focussed on a specific type of rare genetic condition: chromosome 14 deletions. Children with this genetic condition often show developmental delays and brain and neurological problems, although the type and severity of symptoms varies depending on the size and location of the deleted genetic material. The specific aim of the present study was to describe the developmental trajectories of language skills in a group of children with linear 14q deletions. Four children with an interstitial deletion of the long arm of chromosome 14 were followed for 1 year. Data collected from psychomotor and linguistic assessments highlight a large individual variability. Considering the children's genetic and clinical conditions, findings revealed that the size of the deleted area is not related to outcome. However, the developmental trajectories of language development are deeply influenced by the presence of clinical conditions, such as autism spectrum disorders.Clinical Linguistics & Phonetics 12/2012; 26(11-12):962-74. · 0.78 Impact Factor
Article: Prevalence of SHANK3 variants in patients with different subtypes of autism spectrum disorders.[Show abstract] [Hide abstract]
ABSTRACT: Autism spectrum disorders (ASDs) include three main conditions: autistic disorder (AD), pervasive developmental disorder, not otherwise specified (PDD-NOS), and Asperger syndrome. It has been shown that many genes associated with ASDs are involved in the neuroligin-neurexin interaction at the glutamate synapse: NLGN3, NLGN4, NRXN1, CNTNAP2, and SHANK3. We screened this last gene in two cohorts of ASD patients (133 patients from US and 88 from Italy). We found 5/221 (2.3%) cases with pathogenic alterations: a 106 kb deletion encompassing the SHANK3 gene, two frameshift mutations leading to premature stop codons, a missense mutation (p.Pro141Ala), and a splicing mutation (c.1820-4 G>A). Additionally, in 17 patients (7.7%) we detected a c.1304+48C>T transition affecting a methylated cytosine in a CpG island. This variant is reported as SNP rs76224556 and was found in both US and Italian controls, but it results significantly more frequent in our cases than in the control cohorts. The variant is also significantly more common among PDD-NOS cases than in AD cases. We also screened this gene in an independent replication cohort of 104 US patients with ASDs, in which we found a missense mutation (p.Ala1468Ser) in 1 patient (0.9%), and in 8 patients (7.7%) we detected the c.1304+48C>T transition. While SHANK3 variants are present in any ASD subtype, the SNP rs76224556 appears to be significantly associated with PDD-NOS cases. This represents the first evidence of a genotype-phenotype correlation in ASDs and highlights the importance of a detailed clinical-neuropsychiatric evaluation for the effective genetic screening of ASD patients.European Journal of Human Genetics advance online publication, 15 August 2012; doi:10.1038/ejhg.2012.175.European journal of human genetics: EJHG 08/2012; · 3.56 Impact Factor
Article: Replication of association of CHRNA4 rare variants with sporadic amyotrophic lateral sclerosis: The Italian multicentre study.[Show abstract] [Hide abstract]
ABSTRACT: Neuronal nicotinic acetylcholine receptors (nAChRs) are ligand-gated ion channels widely expressed throughout the mammalian brain, including bulbar and spinal motor neurons. They are involved in neuroprotection and in control of release of many neurotransmitters, including glutamate. Previous data raised the hypothesis that rare variants in the region coding the intracellular loop subunits of nAChRs might represent one of several genetic risk factors for SALS. The aim of present study was to replicate the study in an independent cohort of ALS patients. We analysed 718 sporadic ALS patients from five Italian ALS centres and 1300 ethnically matched controls. We focused primarily on CHRNA4, encoding α4 subunit, since most mutations were previously detected in this gene. We observed a significant association between CHRNA4 mutations and ALS (OR 2.91; 95% CI 1.4080-6.0453; p = 0.0056). Most mutations detected in patients were not present in the dbSNP134 and in 3500 ethnically matched control chromosomes and affected evolutionary conserved amino acid residues. In conclusion, the present data confirm that CHRNA4 variants are overrepresented in SALS strengthening the hypothesis can they act as predisposing genetic factors for SALS.Amyotrophic Lateral Sclerosis 08/2012; 13(6):580-4. · 3.40 Impact Factor
Article: Classification of familial amyotrophic lateral sclerosis by family history: effects on frequency of genes mutation.[Show abstract] [Hide abstract]
ABSTRACT: OBJECTIVE: To classify familial amyotrophic lateral sclerosis (FALS) on the base of family history, and to determine whether frequency of mutations in major amyotrophic lateral sclerosis (ALS) genes varies in different FALS categories. METHODS: Included in the study are 53 FALS families. Patients were classified as definite, probable and possible FALS, according to recently proposed criteria. Seven ALS-associated genes, including SOD1, TARDBP, FUS, ANG, ATXN2, OPTN and C9ORF72, were analysed. RESULTS: Thirteen patients (24.5%) were included in the definite group. The great majority of our FALS cases (40/53, 75.5%) were families with only two affected relatives; of these, 31 (58.5%) were included in the probable, and 9 (17%) in the possible FALS categories. The percentage of mutations was 61.5% in definite, 41.9% in probable and 11.1% in possible FALS. With respect to probable FALS, if cases with parent-to-child transmission of the disease were considered separately, the mutational load increased to 61.5%, as observed in definite FALS. CONCLUSIONS: Our findings provide evidence that frequency of mutations in currently known ALS genes varies widely among different FALS categories. Families with only two affected relatives have heterogeneous genetic components, the chance to detect mutations being higher in cases with parent-to-child transmission.Journal of neurology, neurosurgery, and psychiatry 07/2012; · 4.87 Impact Factor
Article: Contribution of major amyotrophic lateral sclerosis genes to the etiology of sporadic disease.[Show abstract] [Hide abstract]
ABSTRACT: To quantify the overall contribution of mutations in the currently known amyotrophic lateral sclerosis (ALS) genes in a large cohort of sporadic patients and to make genotype-phenotype correlations. Screening for SOD1, TARDBP, FUS, ANG, ATXN2, OPTN, and C9ORF72 was carried out in 480 consecutive patients with sporadic ALS (SALS) and in 48 familial ALS (FALS) index patients admitted to a single Italian referral center. Mutations were detected in 53 patients, with a cumulative frequency of 11. Seven of them were novel. The highest frequencies of positive cases were obtained in TARDBP (2.7%), C9ORF72 (2.5%), and SOD1 (2.1%). The overall group of mutated patients was indistinguishable from that without mutations as no significant differences were observed with regard to age and site of onset, frequency of clinical phenotypes, and survival. However, by separately evaluating genotype-phenotype correlation in single genes, clinical differences were observed among different genes. Duration of disease was significantly shorter in patients harboring the C9ORF72 expansion and longer in the SOD1 group. A high frequency of predominant upper motor neuron phenotype was observed among patients with TARDBP mutations. Two patients, 1 with C9ORF72 and 1 with SOD1 mutation, had concurrent ANG mutations. Mutations were detected in 43.7% of patients with FALS. A considerable proportion of patients with SALS harbored mutations in major ALS genes. This result has relevant implications in clinical practice, namely in genetic counseling. The detection of double mutations in 2 patients raises the hypothesis that multiple mutations model may explain genetic architecture of SALS.Neurology 06/2012; 79(1):66-72. · 8.30 Impact Factor
Article: Frontotemporal dementia, Parkinsonism and lower motor neuron involvement in a patient with C9ORF72 expansion.[Show abstract] [Hide abstract]
ABSTRACT: It has been recently reported that a large proportion of patients with familial and sporadic amyotrophic lateral sclerosis (ALS) and/or frontotemporal dementia (FTD) carries the hexanucleotide (GGGGCC) repeat expansion in the first intron of C9ORF72. We describe a patient with a complex phenotype characterized by behavioural variant of FTD, Parkinsonism and ALS with predominant lower motor neuron involvement in which the C9ORF72 expansion was detected.Amyotrophic Lateral Sclerosis 06/2012; · 3.40 Impact Factor
Top Journals
Catholic University of the Sacred Heart
Milano, Lombardy, Italy
- • Institute of Medical Genetics
- • Institute of Neurology
- • Institute of Clinical Pediatrics
- • Faculty of Medicine and Surgery
- • School of Hematology
- • Institute of Pathological Anatomy
Institute of NeurologyMoskva, Moscow, Russia
University of FlorenceFlorens, Tuscany, Italy
Università degli Studi di Milano-Bicocca
Milano, Lombardy, Italy
- Department of Psychology
Università degli Studi di Torino
Torino, Piedmont, Italy
- Dipartimento di Neuroscienze
The Catholic University of AmericaWashington, Washington, D.C., United States
Azienda Ospedaliera Santa Maria Nuova di Reggio EmiliaReggio nell'Emilia, Emilia-Romagna, Italy
ARUP Laboratories: A National Reference LaboratorySalt Lake City, Utah, United States
St. Helena HospitalSaint Helena, California, United States


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Delta Music Institute Opens With Peavey Endowment
CLEVELAND, Miss. â Peavey Electronics has endowed a major musical-equipment grant totaling $60,000 to support curricula at the Delta Music Institute, a center for interdisciplinary studies in the music and entertainment industries at Delta State University.
âThe music of the Mississippi Delta has influenced countless musicians around the world, and it inspired my lifelong passion for making the tools that allow us to create music,â? said Hartley Peavey, founder and CEO of Meridian-based Peavey Electronics Corporation, one of the worldâs largest makers of music and sound equipment. âI hope that this endowment will act as a bridge to link the rich musical legacy of the Delta region to the next generations of music professionals.â?
Music-industry veteran Tricia Walker, director of the Delta Music Institute, said that the Peavey endowment will bolster the programâs burgeoning live audio production department, providing students with a rare opportunity to work with state-of-the-art sound reinforcement technology. Students in the Delta Music Institute demonstrated the gear during its grand opening celebration, held March 7 and 8 on the campus of Delta State University in Cleveland.
âWe are very grateful to receive such generous support from Peavey Electronics,â? said Walker. âAs one of the worldâs largest makers of musical equipmentânot to mention a technology leader with more than 180 patents to its creditâPeavey stepped up with a complete sound-reinforcement package that will be instrumental in our studentsâ success at the Delta Music Institute.â?
The endowment includes a sound-reinforcement system featuring the Peavey Versarray⢠line-array loudspeaker system, the ribbon-loaded, high-fidelity sound system used on the 2008 and 2009 Rockstar® Energy Drink Mayhem Tours and the 2008 Tim McGraw Live Your Voice Tour. The endowment also includes a Peavey 32FX⢠analog mixer with fully digital processing; Peavey QW® and SP⢠Series monitors; Peavey VSX⢠loudspeaker management systems; Feedback Ferret® processors with patented feedback elimination technology; assorted cabling; and an array of microphones that will pull double duty in the DMIâs studio program.
âBuilding on the tradition of Mississippiâs undeniable musical history, this gift will support the creative dreams of the next generation of young musicians,â? added Walker. âThe Delta Music Institute, with its entrepreneurial and interdisciplinary approach to studying the music and entertainment industries, serves to advance the musical culture that is alive and well in Mississippi.â?
Founded in 1965, Peavey® is one of the world's largest manufacturers of musical instruments and professional sound equipment. Peavey has earned more than 180 patents and produces more than 2,000 products, which are distributed throughout the United States and to 136 other countries. Peavey and its MediaMatrix®, Architectural Acoustics®, PVDJ®, Crest Audio® and Trace Elliot® brands and affiliates can be found on concert stages and in more than 5,000 airports, stadiums, theme parks and other venues around the world. To find out more, visit www.peavey.com.
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