text:
By Catholic Online (NEWS CONSORTIUM)
12/14/2012 (2 years ago)
Catholic Online (www.catholic.org)
screen star Gerard Depardieu, who remains in tax exile from his native
France has put his mansion up for sale. According to real estate agents,
Depardieu's luxurious mansion is selling at 50 million euros. The actor
currently resides in Belgium.
By Catholic Online (NEWS CONSORTIUM)
Catholic Online (www.catholic.org)
12/14/2012 (2 years ago)
Published in Celebrity
Keywords: Gerard Depardieu, taxes, France, Belgium, mansion, real estate, Paris
LOS ANGELES, CA (Catholic Online) - Described as a "H�tel Particulier," which is a French term for a grand privately-owned town house, the mansion is in Paris's posh 6th arrondissement.
Listed exclusively by estate agent Daniel F�au, the historic H�tel de Chambon at the heart of the Saint-Germain-des-Pr�s quarter on the left bank of the Seine, is described as a "property of 1,800 square meters with adjoining gardens and terraces, with a secondary living area at the other side of the garden."
Prices per square meter in the highbrow 6th arrondissement often fetch prices of 18,000 euro or more, according to French property site Meilleurs Agents.
Depardieu's home was built in 1820 and extensively renovated by the actor in 2003. The property boasts an extensive garden as well as a swimming pool, which is almost unheard of in central Paris.
The 63-year-old actor has joined a growing list of France's top business figures in relocating to Belgium. France's Socialist government says that it will pledge anyone with an income of over one million euros will be taxed at a rate of 75 percent. There are also plans to hike taxes on "grand fortunes" and large inheritances.
Well over six feet and packing muscle, the bearish star is perhaps best known to English-speaking audiences for his roles in Green Card (1990) and Cyrano de Bergerac (1990). Earning up to two million euros per film, Depardieu also has extensive business interests including wine estates and three Paris restaurants.
While the actor stands to lose big when the new tax rules kick in, French Prime Minister Jean-Marc Ayrault denounced Depardieu's move to the Belgian border village of N�chin as "pathetic."
N�chin, part of the Estampuis municipality in the French-speaking Walloon region of southern Belgium, is in walking distance from the French border.
Nearly 3,000 French people have made the village their own personal tax haven. The people here enjoy lower income taxes and are free from wealth taxes and capital gains taxes on private fortunes, unlike their compatriots a few hundred meters away.
Estampuis Mayor Daniel Senassael said he was not "na�ve" as to Depardieu's motivation for moving to N�chin, but told reporters the actor had "fallen in love with the friendliness, beauty and high standard of living" enjoyed by the other villagers.
Taxes are paid according to the rules of the country of residence in Europe, rather than by nationality. Once installed in Belgium, Depardieu will not have to file tax returns to the French authorities.
St. Mark
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By Greg Goodsell
Actor Gregory Walcott had a long and successful motion picture career. He was inexorably linked in what is popularly considered the worst film ever made, "Plan 9 from Outer Space," directed Edward D. Wood Jr.'s confused opus about space aliens attempts to bring ... continue reading
By Catholic Online (NEWS CONSORTIUM)
That's A LOT of toilet paper... manic comedian Howie Mandel, who suffers from Obsessive Compulsive Disorder, was away in New York City, leaving his California mansion - vulnerable. Roman Atwood of "Deal or No Deal," pulled off an elaborate prank. With the ... continue reading
By Catholic Online (NEWS CONSORTIUM)
By Catholic Online (NEWS CONSORTIUM)
Actor Robert Downey Jr. plays superhero Tony Stark, of the wildly successful "Iron Man" movie series. A little bit of his onscreen superhero must have rubbed off on Downey in real life. He recently gifted a seven-year-old boy, who was born with a partially ... continue reading
By Catholic Online (NEWS CONSORTIUM)
A hero in real life as well as the movies, actor Harrison Ford is expected to recover after crash landing a World War II plane to safety on a California golf course. LOS ANGELES, CA (Catholic Online) - Landing the plane on a fairway at a municipal course in the Los ... continue reading
By Hannah Raissa Marfil (NEWS CONSORTIUM)
American superstar, Taylor Swift, surprised a four-year-old, terminally ill girl with a call via FaceTime earlier this month. Swift's chat with Jalene Salinas, that lasted about 20 minutes, gave Jalene a renewed strength to continue battling her terminal brain tumors. ... continue reading
By Troy Dredge, Catholic Online
Even if you weren't a "Trekker," one who obsessively follows the TV series "Star Trek" and all its permutations over the years, there was no escaping the character of Mr. Spock. A member of the Vulcan race, Leonard Nimoy played the gaunt-faced, pointy eared and ... continue reading
By Catholic Online (NEWS CONSORTIUM)
The role of a voiceover artist is typically an anonymous one. Such was not the case for radio disc jockey and talk show host Gary Owens. Suave and debonair, one hand covering his ear, Owens' rich baritone speaking voice made him a natural for repeating inane ... continue reading
By Catholic Online (NEWS CONSORTIUM)
His name raises ire among all so-called serious practitioners of "serious literature." Talk show host Dick Cavett once declared Rod McKuen as "the world's most understood poet." From his most famous poem, "Listen to the Warm:" "You have to make the good times ... continue reading
By Troy Dredge, Catholic Online
American illustrator Edward Gorey, who died in 2000 at the age of 75, left behind an endearing legacy of "not quite right" storybooks enjoyed by children and adults. His stories were set in a gloomy netherworld that called to mind Victorian England, Edwardian ... continue reading


text:
- Exchange: Tel Aviv
- Sector: Industrials
- Industry: Engineering & Construction Svcs
- Sub-Industry: Infrastructure Construction
Baran Group Ltd+ Add to Watchlist
BRAN:IT2,095.00 ILs 0.000.00%
As of 10:24:54 ET on 03/31/2015.
Company Profile for Baran Group Ltd (BRAN)
Baran Group Ltd. provides engineering, building, management and manufacturing services for construction and industry. The Company also develops and manufac- tures technology products and compounds.
Contact Information
Baran Group Ltd
8 Omarim Street
Omer, 84965
Phone: 972-08-620-0200
Fax: 972-08-620-0201
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|Dor MeirChairman
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|Steven SenterDivisional CEO:Baran International
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|Arik ShakedVP:Business Development
|Issac FriedmanGM:Infrastructure & Construcion
|Sasson ShiloSenior VP:Finance & Audit
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subset: commoncrawl


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VIEWS: 12 PAGES: 7 CATEGORY: Business POSTED ON: 2/23/2010
All I Need Is The Air That I Breathe
"All I Need Is The Air That I Breathe" For those of you who joined the scouts as a youngster the phrase "Triangle of Fire" should ring a bell. This mnemonic states you need three things to start a fire; fuel, air and a little heat. The internal combustion engine uses the same principle. You need petrol, air and a spark to make things happen. I will not be touching upon petrol or sparks. Instead I will be concentrating on air. Engines, like most animals, need to inhale and exhale air. I won't be talking about exhalation either (the exhaust system). I will however focus on inhalation, or to give it its proper designation, the induction system. Induction is the means by which the engine inhales air. Simply put - the more air available the better. One could go to the extent of adding a turbocharger or a supercharger both of which force air into the engine. But I for one don't have two or three grand to spare! Now if only I could find a way of shortening the air intake tract so the air had less distance to travel to the engine. Perhaps I could reduce any resistance the air may have to overcome on the way. I might be able to use the forward motion of the car to ram air into the system. And maybe I could get colder air to the engine, which means denser air, which means more air – that would be good too (for every seven degrees Fahrenheit of cooler air you can add about one percent of power). You see, the stock induction system has a long intake tract. The standard air filter slows down incoming air. The system takes air from inside the engine compartment, which generally means hot air. I could be onto a winner here. But no, hold on, Jackson Racing has beaten me to it. Their "Cold Air Induction System" addresses all my points. The intake tract is about half the length, it employs a high flow air filter, it takes cool air from in front of the radiator and the forward motion of the car helps drives this air into the system. And so, distraught at someone else pinching my great idea, I dusted the cobwebs from the credit card, parted with just over £340 and waited for the system to arrive in the post. My father, a retired motor mechanic, provided the necessary garage facilities and supervision. What follows is a brief description of how the installation went. We began by removing the existing air intake tract from where it begins, at the near side front wing, all the way across to where it joins the throttle body on the offside of the engine. This whole section is quite long - perhaps four feet or more of piping. It incorporates the air box, air filter and air flow meter. There were no problems encountered during this stage – simply a matter of undoing the usual nuts, bolts and screws. The air flow meter would be set aside for use later. Next, the top water hose coming out from the thermostat housing would need to be raised to allow room for the new intake tract. An aluminium spacer provided the extra height. This process involved removing the top hose, removing the top of the housing and inserting the spacer (gaskets provided). We left the top hose off until the very end of the installation as this afforded us more working space. Then we bolted the old air flow meter onto the side of the new air box. An extension cable came with the kit that enabled the meter to be reconnected to the computer. Next, we built up the new tract bit by bit. A black "elbow" section fitted on to the throttle body. A concertina type flexible tube fitted on to that. The other end of this tube fitted onto the open end of the old air flow meter. Two bolts on the front cowling secured the main air box in place. Our very own STHT editor, Clive, suggested I use rubber washers underneath the supplied metal washers to prevent the plastic air box suffering from fatigue cracks. We actually went one stage further. By enlarging the mounting holes on the front of the air box we were able to insert rubber grommets. We then inserted some short metal tubing through the centre of the grommets. This allowed us to securely fix the front of the air box whilst cushioning any movement. Quite a nice solution (thanks Dad). We refitted the top hose (topping up the system with anti-freeze). On doing this it seemed the hose was very close to the top of the relocated air flow meter. We filed down a very small section of ridging from the top of the meter and this gave a little more space. Finally, my car has a brace across the engine bay so the suggested new site for the bonnet support was not appropriate. We improvised. We relocated the base of the support as the instructions suggested. However, a couple of carefully placed bends in the support enabled it to zigzag between the new air box and the radiator filler cap and still fit back nicely into the original snap-in holder. We spent about two hours thirty minutes on this job. I reckon, having done it once, we could easily get this down to one hour thirty minutes or maybe even less. And the result - the stock 1.6L Mk1 gives out 116 bhp and propels the car from 0 to 60 mph in 9.1 seconds. Before the installation I measured my car on a rolling road at 131 bhp and my 0-60 time was 8.4 seconds (interestingly the same as a stock 1.8L model). After the installation power had jumped to a quite satisfying 144 bhp, that's 13 bhp more, about a ten percent increase - and you can tell – 0-60 in 7.9 seconds! 2/11/3 Harry Baldwin Worcester Member in the South West Midlands Region
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Dexter Russell Narrow Fillet Knife
High-quality knife at an affordable price
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Robert Sage
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Donny Aultman
Mary L. Burgess
My great aunt, thanks for posting the photo.
Curious about that burial date? DOD 1978, Burial 1979?
Is there a story there?
Added by
Donny Aultman
on Mar 28, 2015 9:48 AM
Steve Burton
RE: web site
Robert, It looks good to me, no parents and no sibs. Again I apologize.
Best Wishes,
Added by
Steve Burton
on Mar 22, 2015 9:23 PM
Steve Burton
Sarah Allen Hauer
Thanks so much for the transfer of Sarah Hauer. It's nice to have a little piece of her.
Best Wishes,
Added by
Steve Burton
on Mar 22, 2015 4:48 PM
Steve Burton
RE: Margaret Hofer
Hi Robert,
Thanks for getting back to me.
I sure could be wrong. My experience with Thelma, as we called her, was that she married Giles as husband number 1. Then she abandoned her 4 children and ran off with husband number 2. I really don't have it clear what his surname was, but I think I can find out. Thelma's grandson is on ancestry and a cousin of mine. Maybe he will know.
If Thelma were Margaret's mother, Thelma would have been 15 at the time of birth. ? I don't know.
Let me get back to you about husband number 2's surname.
Thanks, Robert.
Added by
Steve Burton
on Mar 21, 2015 4:24 PM
#55950073 - Wm Starkey
Dear Robert,
Thank you so much for adding the photo of William Starkey. Where did you find it?
Added by
on Mar 16, 2015 3:24 PM
Gaylon Powell
James Norman Smith
Thank you so much for the transfer of James Norman Smith.
Added by
Gaylon Powell
on Mar 08, 2015 1:16 PM
KD Burleson
RE: Spec Robert Earl Cole
Your welcome, I usually take the time if it involves one of our fallen soldiers. His monument had sunken down, so I repositioned it as best I could for the photo.
Added by
KD Burleson
on Mar 06, 2015 4:03 PM
Harry J Traphoner, Jr
Curious, where did you get the photo of my great uncle Harry J Traphoner, Jr? Are you related to him?
Added by
on Mar 06, 2015 2:45 PM
D Ulczynski
Regina B. Gerling
I would appreciate having Regina B. Gerling #79995821, my paternal grandmother, transferred to me to manage.
Debbie Gerling Ulczynski
Added by
D Ulczynski
on Mar 02, 2015 3:50 PM
RE: Inquiry into J Burns
Awesome! Thank you.
It is interesting that she died of consumption. I believe this is the same woman who travelled to what was known as Wynne at the time in the 1883 - 1884 time frame, now known as Nazareth. She gave birth and the baby died. It was buried in what has become an unmarked grave somewhere around Nazareth. She died, but it is not listed in the records how she died. She was only known as Mrs. John Burns. Her body was transported back to Amarillo where it was then transferred to Austin for burial.
Added by
on Feb 25, 2015 8:46 PM
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Interior Trim Kit
Reviewed By Laura H (Mechanicsville, VA) —4/22/2012 10:22 AM
Great! I gave it to my boyfriend as a birthday gift and he loves it. Easy to install and adds a lot to the plain black interior
At the 2012 SEMA Show, Business Development Manager for Rugged Ridge, Pat Johnson showed off some new Rugged Ridge products.
Nothing screams used car like a faded, cracked and warped dashboard. But, when you set your ride up with a new dash kit, you can revert your vehicle into a stylish, clean new car.


text:
National Junior Ranger Day Saturday
Published: Monday, April 21, 2014 at 4:48 p.m.
Last Modified: Monday, April 21, 2014 at 4:49 p.m.
Little River Canyon National Preserve will celebrate National Junior Ranger Day on Saturday in conjunction with Russell Cave National Monument, DeSoto State Park and the Little River Canyon Center.
Junior Ranger materials from all three parks will be available at the Cornbread Festival in South Pittsburg, Tenn., and at the parks.
Young people can earn a DeSoto State Park Junior Ranger patch or Little River Canyon or Russell Cave Junior Ranger badge by completing the booklets.
This is the fifth year Little River Canyon has had special Junior Ranger programs at the Little River Canyon Center.
The Junior Ranger programs provide educational activities for young people in national and state parks with rewards of patches, badges or certificates.
For more information, call 256-845-9605.
Reader comments posted to this article may be published in our print edition. All rights reserved. This copyrighted material may not be re-published without permission. Links are encouraged.


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Mizzou's 2014 season opener vs. South Dakota State will be televised nationally by ESPNU at 2:30 p.m. on Saturday, Aug. 30.
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7 of 7 people found the following review helpful
Dark and Engrossing True Story French Film,
Verified Purchase(What is this?)
This review is from: Our Children [DVD] (DVD)
Based on true events, this French film tells a very dark tale indeed. It is about Andre (Niels Arestrup `War Horse' and `A Prophet') who plays a doctor who seems to have a soft spot for Algerians who want to escape to France to have a better future. He has married one for her convenience and then he sponsors her brother Mounir (Tahar Rahim `Free Men' and `also `A Prophet'). They seem to have a very close bond indeed so when Mounir announces he wants to marry a French woman, there is a moment of awkward friction.
This woman is school teacher Murielle played by the achingly beautiful Emilie Dequenne (`The Pack' and `Brotherhood of the wolf'). They waste absolutely no time in starting a family and Andre insists that they live with him. He sees himself as part of the larger family and what at first seems to be a loving and helpful man soon starts to be revealed as a manipulating and very controlling person indeed. As his influence grows so does the gap between the erstwhile love birds and it is only a matter of time before things will come to a head.
Director Joachim Lafosse has managed to make a harrowing true story be very watchable and engrossing with his portrayal of the inter relationships that make people act in a way they would never have meant to. All of the performances are excellent with Emilie Dequenne putting in an exceptional performance of a woman slowly coming unravelled; she seems to age as the film progresses. Issues around illegal immigration are touched upon but the morality is left for the viewer to decide. This was a co-production of companies in France, Belgium, Luxembourg and Switzerland and is a case where too many cooks can make a rather excellent meal of a film altogether. This is no feel good film either, but with the subject matter it was never going to be. In French and Arabic with very good sub titles and a run time of 111 minutes all of which are put to excellent use - this is a film for lovers of European cinema and fans of Niels Arestrup who is also excellent in the slow burning role of a controlling dictator - highly recommended.
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text:
Stripping a painted fireplace - how to
I posed this question several months ago when I first began researching (...and obsessing) about this project. There doesn't seem to be a lot of information out there (perhaps because it's an annoying and tedious task...).
But, as one of those who have tackled this and come out the other side, I thought I would share some tips and tricks and pics regarding the process. I won't post every picture, but I have documented the entire process.
Keep in mind: "YMMV!" This is one of the most important factors I learned with the process.
I found some info here and elsewhere. I posted questions at multiple DIY sites. I even called the American Brick Union for advice which was 'never sandblast'.
Some DIYers said they were able to complete this project in 30 days or less. Oh - really? It can't be that bad, right? Again - YMMV.
It took me about 4 and a half months from start to finish. This isn't working on it all day every day. You will loose your mind stripping brick 'all day, every day'. You have to walk away to keep your sanity as your friends will constantly be questioning if you have lost your mind by even attempting this task.
Also, it's a very physical process - my arms were quite tone and well muscled after this was done. My knees hurt...so get a good stool to sit on.
What matters is the type of paint on the first surface and then, how many layers of paint in total and the TYPE of paint on each revealed surface. As each layer of paint is removed, you're going deeper into time when oil based-lead paint was used or milkpaint, etc.
This requires different types of products in order to effectively strip the paint. It's not a 'one remover for all' situation. I used 2 types of paint stripper: Peel Away 7 for the first layers of latex paint and Peel Away 1 for the remaining layers oil based/lead paint - in that order due to the type of paint on the brick and both were recommended by PeelAway for masonary. I did speak w/PA several times for assistance. They were very helpful and even sent me a free sample of Peel Away 1 to test.
In my initial start, I discovered that there were 9-10 layers of paint. The original being the most difficult to remove - an ugly, mint green oil (?) based paint.
Even at the end of the process, I was not able to remove ALL paint. Paint over years somehow embeds into a porous product like brick to the point where full removal may be impossible.
Starting the process...fireplace is currently painted an off-white that just looks 'dirty' all the time.
A very important point on floor protection. I have lovely hardwood floors that I didn't want to damage in the process. Again - YMMV - but, through trial & error, I discovered that using layers helped the most.
First, layer thick and many, many towels, then, plastic paint drop sheeting, then thick layers of newspaper. I also took paint drop sheeting and taped it up the walls to prevent the walls from being flicked with paint removal particles.
It's goopy and runny and scrubbing brick with wire brushes is a dirty, dirty job and sprays/flicks paint chunks every where - walls, floors, your hair....every where...wear goggles/eye protection for this process.
It takes lots of buckets of water to rinse Peel Away off. For PeelAway 1, you also have to rinse with vinegar at the end. ALOT of vinegar! I used about 14-20 gallons of vinegar. I learned that the hard way when I began cautiously sprayer vinegar and it didn't do much. So, I filled a bucket with it and used it to SCRUB the brick. HUGE improvement and right away.
None of the PeelAway products had awful odors and I had no problems with it in the house for months. It is not a 'quick' product due to it having to sit for 24hours and sometimes a second application is required. But, I was pleased with how it worked overall. Oh, it really doesn't come off onto the paper when you are applying this to brick. Don't waste your time being frustrated by that. Still cover it up with plastic sheeting to 'cook', but, you'll be scraping the goop off with plaster scrapers the next day.
Another tip, purchase a wide variety of wire brushes, plastic brushes, scraping tools, picks, grout cleaners and use your imagination on creating what you need. You'll go through a lot of brushes due to scrubbing brick.
Again, some of this required multiple applications of PeelAway (PA). You can see the difference in some prior pics. This was the point that I was wondering if it would ever look good and if I was wasting my time doing this?...all that work and it wasn't looking "lovely" by any means...major panic happening!
Close up of brick. The top row had a second treatment...the bottom did not. Btw, failed to mention, my brick has a rough-cut brickface. Nothing smooth about it. If it were smooth, it would probably be a much easier process.
With white vinegar and heavy scrubbing/rinsing...you can see the big difference!
Done with the stripping!!!
Applied several coats of 50/50 BLO (Boiled Linseed Oil) and mineral spirits. Really brings the color of the brick back. I hate the smell of BLO personally....eventually it does dissipate.
Here it is - done. For the most part, anyway. I still need to stain the mantle and trim. I'd like to touch up some of that brick too to remove some of the paint that is still showing. It's not 'perfect' but I'm happy with the outcome and do not regret taking the time to do this hard job. Although, during this process, I definitely had my 'second thoughts', but was too invested to stop.
I hope those of you who are contemplating this job find viewing my experience helpful!
In the process, while I was working on the fireplace, I also replastered the wall and stripped the window trim which still needs repainting. So, there is still more tasks to be tended too.
Excellent job, saintpfla! I'm one of those who posted at your start and I said 1 month. That's what it took me....on smooth brick! I'm facing another painted fireplace....this time with the same rough brick you tackled. I've really dragged my heels because of the texture. Now you are MY motivator! Get through the holidays, get the tax stuff in order and then I tackle it!
Hi Lido!
Yes - I remember your encouragement very well and it was much appreciated! I do recall the 'one month' part and I kept thinking....'why is this taking me so darn long!!!!'
Perhaps I can return the favor to you and offer some advice as well. I do have more pics if you need to view any for your research before you try to tackle the job.
The textured brick is a TOUGH job to strip. Unless you discover some trick that I have not found, just forget about removing all of the paint from each crevice - I don't think it's even possible. Mine has somewhat of a rustic/distressed look due to the paint residue.
I described this project to my friends as, "....the equivalent of trying to remove paint from an English Muffin."
I am just glad that I was successful in not ruining my hardwood floors - yaaay!
Great job. Our house had a few coats, one white, and two to simulate the look of brick and mortar. I used peel away as well. I think it too me two applications and I came to the reaization that there is no removing the white spots in the rough brigs.


text:
Lol, this place is as far from a political forum as one can get. My issues isn't with the facts of sales data. That's irrefutable. My discussion begins at the debate that the system war is already over and there is no hope for the One. That's my discussion. The One has sold very well if looked upon based on the 360's launch.
Being far more expensive and in far less territories than its competitor. But the PS is having monster sales and undeniably is ahead worldwide. No argument there irregardless of my own personal opinion of the system. Strong sales are facts. I recall the 360 bashing the PS3's head in for years and how SW was then, so its no different now with the PS4 laying the smack down, lol :)
The original xbox sold more than the xbox 360 in its first 4 months yet the 360 sold 3 times more,the problem with the 360 is simple it was very supply constrained because of yield issues and flow of units didn't improve until April or more of 2006,so is easy to beat the 360 numbers.
Oh Please $100 more,and since late February is been sold with a free copy of its hottest and most hyped game,worse since March 21 retailers like walmart had been selling it for $449 with Titanfall which is $10 less than a PS4 with a game.
Have you wonder why MS instead of letting those xbox one collecting dust in retailers storerooms,they didn't ship them to the so call countries that were left out.?
Is simple those countries will not add anything significant to MS,in fact outside UK the xbox one on EU is been outsold like 4 to 1 by the PS4,and those were MS best markets,so the worse ones they will sell even less.
Quite a psycic you are there :) Its 5 months into this thing and nothing is absolutely for certain. To say otherwise is pretty naive. MS and Sony both sold 80+ million last gen, and the PS4 is at 7+ and the One at 4+. That's a shitton of people who are waiting to buy their console. Both will enjoy price cuts and sell very well. MS needs a price cut, while Sony if they get undercut can match it. But price and games sells systems, and the One will get a price cut and games. There's time to turn this around for certain. That's not a guarantee. But to say who will sell what, where, for certain, when it isn't even released there yet doesn't make sense to me.
As for those systems not sent to countries that the One hasn't released in yet, the One does a hell of a lot more than gaming and certain things aren't perfected or work in those regions yet. Better to release a product when you know its ready And then bust your ass to convince people its good then sell it now when its broken or vastly unfinished.


text:
Olivia Newton-John, whose new wedding-themed comedy A Few Best Men will open in the spring, has been a longtime favorite of LGBT fans and a supporter of marriage equality. In July, the singer spoke to The Advocate about her experience performing at New York's gay pride the night after same-sex marriage was legalized in the state. "The air was electric, and there was so much excitement in the air from the couples," she said. "It's wonderful that it can be recognized. People who have had long relationships and care about each and take care of each other should have the right to be married." Watch her perform "Have Yourself a Merry Little Christmas" below.
© 2015, Here Media Inc. All Rights Reserved


text:
*from and organic source **all fragrances naturally derived.
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Dictionary » A » Air thermometer
Air thermometer
Air thermometer
See: gas thermometer.
Please contribute to this project, if you have more information about this term feel free to edit this page
Results from our forum
HELP: Growing Bacteria
... 1) How long would it take me to grow the E. coli without an incubator? I have a heating pad, and after setting it on LOW, I wrapped it around a thermometer. According to the thermometer, it's 35 Celsius when wrapped. So, could I wrap the E. coli petri dishes with a heating pad on low setting ...
See entire post
Re: Legitimate Global Climate Change Skepticism?
... world, on average warms at 0.08 C per decade. That is a simple fact. Arctic regions warm at 3 times that rate. Also a simple fact. These come from thermometer readings, not political speeches. Over geologic time, sea levels rise and fall. So does temperature. In the last interglacial period, 120,000 ...
See entire post
Finding the amount of energy in a certain food question
... part c, as I wished to write, what bad experiment it is. Of course, you will lose energy, as you do nt have it isolated, so you should use some thermometer or how is it called... Regarding e), look for what they mostly contain (saccharides, lipids, proteins)
See entire post
Re: How do Cells "think"...
... know if the temperature was rising steadily (pretend that could mean some sort of danger). The "feedback" that the refer to allows the thermometer to "detect that the temperature has risen" - then reset itself so that the current temperature is "now the norm and forget ...
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How to be "good at the bench"
... and will always be a catastrophe in the lab (there is such a master student in our lab, but last month he broke plenty of glassware, a mercury thermometer and a 3000$ piece of equipment...)
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text:
clamping the barrel in a vise makes a big difference! should come out with a punch and hammer.
Just walking on the edge of of my grave.
OUCH!! We were deer hunting one year when one of my buds said his gun was shooting way to the right. All we had was a hammer & screwdriver to perform this operation. No problem, the gun has been through many hunts and it showed it. He held the gun & I proceeded to knock the sight blade and bead plum off. I gave him my back up gun to hunt with. I bought the sight pusher and replacement front sight as soon as I got home. That was my learning experience.
I'd do more fishing if I could use a gun!
The sight has to come out from left to right and go back in from right to left. I always used a piece of hardwood dowel so I would not mar anything.
Patron Member NRA
"I would not be an old man if I had not been an armed young man." JTJ
I was taught to respect my elders but they are getting harder to find.
Get a bigger hammer lol (joke guys). But realy ive had hit some alot harder then u would think to get them moving.
There is always a side-grinder............It is amazing what you can do with one of them if you have a delicate touch.
"It is better to be too skeptical then too credulous"
Carl Sagan
Hint to gunsmiths- - don't let customers see you removing sights with a side grinder.


text:
Legal Question in Landlord & Tenant Law in Florida
I moved into an apartment with my 19 years old daughter and 1 year old grandson. They told me my daughter had to put her name on the lease since she was visiting from Jamaica. Now I got an eviction notice and my daughters name is on it... Is that possible?
2 Answers from Attorneys
Yes it is possible since you added her name to the lease.
From Byron Petersen
If it is a straight eviction action/tenant removal (no claim for rent associated with it) it really does not matter as long as you are on the lease.
You should, however, consider asking the County Court Eviction Judge to remove your daughter from the lease and from the law suit because she did not have to be on the lease when she was a guest and you were improperly forced to add her. Though your daughter may not have any "liability" concerns, for future credit purposes you do not want a judgment against your daughter. She needs to be let out.
Eviction actions have very strict requirements (both procedural and substantive). For example- 5 days to respond to complaint for eviction. Be sure you meet all deadlines.
You might also wish to speak to an attorney at Legal Services in your county.
The above is based on Florida Law and should not be relied upon without a full airing of all circumstances and options with a Florida Lawyer of your choice. Byron Petersen
Related Questions & Answers
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For the last year I rented a house with three roommates (one of the roommates... Asked 8/22/13, 11:08 pm in United States Florida Landlord & Tenants


text:
ReadyNation's 1,000+ members work to strengthen business and the economy through
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is this battery compatible to 13¨Macbook 1,1 Model No.A1181?
I bought it on 2006
- Asked by Chris V from Mexico
- May 12, 2014
QA Rechargeable Battery 13" MacBook
Product No Longer Available
1 Answer from the Community
It is, I have a 13" Macbook 1,1 and have just put a new battery in, this is the one for your Macbook.
- Answered by Tobias B
- May 28, 2014


text:
Page 1
Mutation analysis of NPHP6/CEP290 in patients with Joubert
syndrome and Senior–Løken syndrome
Juliana Helou, Edgar A Otto, Massimo Attanasio, Susan J Allen, Melissa A Parisi, Ian Glass, Boris
Utsch, Seema Hashmi, Elisa Fazzi, Heymut Omran, John F O'Toole, John A Sayer, Friedhelm
............................................................... ............................................................... .....
J Med Genet 2007;44:657–663. doi: 10.1136/jmg.2007.052027
Background: Nephronophthisis (NPHP) is an autosomal reces-
sive cystic kidney disease that constitutes the most common
genetic cause of renal failure in the first three decades of life.
Using positional cloning, six genes (NPHP1-6) have been
identified as mutated in NPHP. In Joubert syndrome (JBTS),
NPHP may be associated with cerebellar vermis aplasia/
hypoplasia, retinal degeneration and mental retardation. In
Senior–Løken syndrome (SLSN), NPHP is associated with retinal
degeneration. Recently, mutations in NPHP6/CEP290 were
identified as a new cause of JBTS.
Methods: Mutational analysis was performed on a worldwide
cohort of 75 families with SLSN, 99 families with JBTS and 21
families with isolated nephronophthisis.
Results: Six novel and six known truncating mutations, one
known missense mutation and one novel 3 bp pair in-frame
deletion were identified in a total of seven families with JBTS,
two families with SLSN and one family with isolated NPHP.
decades of life.1Using positional cloning, six genes (NPHP 1–6)
have been identified to date,2–8causing disease variants NPHP
types 1–6, respectively. NPHP can occur with isolated kidney
involvement or in combination with diverse extrarenal mani-
festations. Specifically, it can be associated with cerebellar
vermis dysplasia/hypoplasia, retinal degeneration and mental
retardation in Joubert syndrome (JBTS).8–10NPHP may also be
associated with retinal degeneration in Senior–Løken syndrome
(SLSN) in about 10% of patients with mutations in NPHP1, 2, 3
or 44 5 11 12and in .90% of patients with mutations in NPHP5 or
6.7 8
JBTS is an autosomal-recessive developmental disorder with
multiple organ involvement characterised by a typical neuro-
radiological feature, the ''molar tooth sign'' (MTS), which
describes a cerebellar and brainstem malformation. This entails
dysplasia or hypoplasia with or without dysplasia of the
cerebellar vermis, thick and misoriented superior cerebellar
peduncles and an abnormally deep interpeduncular fossa.10 13
Accompanying neurological symptoms of JBTS include neona-
tal hypotonia, transient abnormal breathing patterns in the
neonatal period (apnoea and/or hyperpnoea), ataxia, nystag-
mus,mental/psychomotor retardation
apraxia.10 14 15Seizures and behavioural problems within the
autism spectrum have been described.10JBTS and JBTS-related
disorders (JSRD), as defined by the presence of MTS with
multiorgan involvement, are clinically heterogeneous.13Two
forms of JBTS have been determined: types A and B, the latter
being characterised by the co-occurrence of ocular and renal
features, namely retinal dystrophy and nephronophthisis.14At
ephronophthisis (NPHP) is an autosomal recessive cystic
kidney disease, which is the most common genetic cause
of end-stage renal disease (ESRD) in the first three
and/or oculomotor
least eight distinct syndromes that have MTS as a feature have
been reported, showing wide phenotypic variability both within
and between families.13One example is COACH syndrome15in
which cerebellar vermis hypoplasia occurs along with oligo-
phrenia, congenital ataxia, ocular coloboma and hepatic
fibrosis. Additional extrarenal organ manifestations may
include retinal coloboma, congenital amaurosis, hepatic fibrosis
and postaxial polydactyly. MTS has also been described in
association with complex midbrain–hindbrain malformations
including Dandy–Walker malformation15and occipital ence-
phalocoele.10 13
JBTS is genetically heterogeneous. Four genes have been
identified and two additional loci mapped for JBTS,16including
the JBTS1 locus at chromosome 9q34.3 (OMIM 213300)17and
the JBTS2 or CORS2 locus at chromosome 11p12-11q13.3
(OMIM 608091).18The gene defects identified in patients with
JBTS include: (1) deletions in NPHP1 (JBTS4; OMIM 607100);10
(2) mutations in the AHI1 (Abelson helper integration site) gene
(JBTS3; OMIM 608894),10 19–21and (3) mutations in the MKS3/
TMEM67 (Meckel syndrome type 3) gene (JBTS6) which was
initially found in patients with Meckel–Gruber syndrome
(OMIM 607361).22In addition, using positional cloning, we
recently identified mutations in a novel gene NPHP6/CEP290
(OMIM 610142), as causing JBTS5,8describing eight different
mutations in seven families with JBTS.8Mutations in the same
gene were identified in five families with pleiotropic forms of
JBTS by Valente et al9and as a major cause of JBTS with
oculorenal involvement by Brancati et al.23Recently, a hetero-
zygous truncating mutation in NPHP6/CEP290 anda heterozygous
missense mutation in AHI1 have been found in combination with
the homozygous NPHP1 deletion in patients with NPHP and
JBTS-related neurological symptoms.24
The most common extrarenal manifestation of nephro-
nophthisis is retinal involvement. In SLSN there is the
association of NPHP with retinitis pigmentosa, tapetoretinal
degeneration or retinal dysplasia.1About 10% of patients with
mutations in NPHP11or NPHP46present with SLSN. In one
patient with SLSN, a mutation in NPHP2 was identified.12In
NPHP3, only single heterozygous mutations were found in four
patients with SLSN.4Whether a mutation in an unknown
modifier gene is causative for the retinal involvement is
unknown.1In NPHP5, all patients described to date have had
early-onset SLSN.7Recently we reported a homozygous 5 bp
deletion in NPHP6/CEP290 that altered an obligatory splice site
in a patient with SLSN.8
Another condition that can occur concomitant with NPHP is
Leber congenital amaurosis (LCA; OMIM 204000) a severe
Abbreviations: ESRD, end-stage renal disease; JBTS, Joubert syndrome;
JSRD, Joubert syndrome-related disorders; LCA, Leber congenital
amaurosis; MTS, molartooth sign; NPHP, nephronophthisis; OMIM, Online
Mendelian Inheritance in Man; SLSN, Senior–Løken syndrome
Page 2
retinal dystrophy causing blindness or severe visual impairment
at birth or within the first 2 years of life. Mutations in nine
genes (AIPL1, CRB1, CRX, GUCY2D, IMPDH1, RDH12, RPE65,
RPGRIP1 and NPHP6/CEP290) have been found to cause
LCA.25 26A hypomorphic mutation of NPHP6/CEP290 in humans
represents the most common cause of LCA.25 26This intronic
NPHP6/CEP290 mutation (c.2991+1655ARG) accounts for 21%
of LCA cases. It creates a strong abnormal splice-donor site
leading to an insertion of a cryptic exon in intron 27 of the
CEP290 messenger RNA.25Similarly, an in-frame deletion in the
orthologue of the gene NPHP6/CEP290 identified in the mouse
mutant rd16 causes retinal degeneration without renal or
cerebellar involvement.27The high frequency of NPHP6/CEP290
mutations in patients with LCA was confirmed by Perrault
et al.26
Thus, mutations in NPHP6/CEP290 have been found in
disorders with cerebello-renal, cerebello-oculo-renal, cere-
bello-retinal, retinal-renal and retinal phenotypes.9 11 23 25 28To
date, mutations in NPHP6/CEP290 had not been identified in
patients with isolated nephronophthisis. In this study we
performed mutation analysis in a worldwide cohort of 195
families with NPHP, SLSN or JBTS. We examined all translated
exons and adjacent intronic sequence and intron 27 for
mutations in NPHP6/CEP290. We found seven novel mutations
(six of them being truncating mutations), in four families with
JBTS, two families with SLSN and one family with isolated
We performed mutational analysis in a worldwide cohort of 195
families with JBTS, SLSN or isolated NPHP. Our cohort
comprised 99 families with JBTS (5 with diagnosis of LCA),
75 families with SLSN (6 with LCA and NPHP) and 21 families
with isolated NPHP.
We collected blood samples, pedigrees, clinical information
and informed consent (www.renalgenes.org). Approval for
studies on human subjects was obtained from the University
of Michigan institutional review board.
In all patients the diagnosis of NPHP was based on one or
more of the following criteria: (1) clinical course with
characteristic clinical signs of polyuria, polydipsia, anaemia
and growth retardation; (2) presence of chronic renal failure;
(3) renal ultrasound or renal biopsy compatible with the
diagnosis of NPHP as judged by a (paediatric) nephrologist; and
(4) pedigree compatible with autosomal recessive inheritance.
Neurological criteria for JBTS were based on the following
clinical hallmarks of this cerebello-oculo-renal syndrome: (1)
MTS or (2) diagnosis of JBTS by a (paediatric) neurologist or
geneticist. Associated JBTS symptoms were recorded: optic
nerve or retinal coloboma, tapetoretinal degeneration, cerebel-
lar vermis aplasia/hypoplasia, ataxia and periodic apnoea/
tachypnoea. The diagnosis of SLSN was based on the presence
of NPHP in association with tapetoretinal degeneration.
Analysis of NPHP1-5, AHI1 before analysis for NPHP6/
Genomic DNA from peripheral blood samples was extracted by
standard methods. Before mutation analysis for NPHP6/CEP290
described in this study, the homozygous NPHP1 deletion and
mutations in NPHP5, both known to cause SLSN and LCA, were
excluded in patients with eye involvement. All patients with
JBTS were tested for the homozygous NPHP1 deletion and for
mutations in AHI1. All patients with isolated NPHP were tested
for the homozygous NPHP1 deletion.29To exclude mutations in
other known NPHP genes prior to this study, 40 patients with
infantile NPHP were tested for mutations in NPHP2,2950
patients were tested for mutations in NPHP34 29and 95 patients
of the cohort were screened for NPHP4 mutations.28 29
NPHP6/CEP290 mutation analysis
In total, 195 samples underwent NPHP6/CEP290 mutation
analysis. Intron 27 and all 54 translated exons of NPHP6/
CEP290 were amplified by PCR using 51 exonic flanking
primers. Initially, all amplicons were prescreened by hetero-
duplex formation and a subsequent CEL I endonuclease digest
as described previously.29The CEL I enzyme recognises single-
base mismatches present in heteroduplex DNA and cleaves
both strands. Mutations can be detected with a sensitivity of
92%.29 30Samples showing aberrant bands in agarose-gel
electrophoresis were purified and directly sequenced. For each
mutation, 94 healthy control individuals were examined by
restriction-enzyme digest or CEL I endonuclease assay.
We analysed a cohort of 99 families with JBTS, 75 families with
SLSN and 21 families with isolated NPHP for mutations in
NPHP6/CEP290. Using heteroduplex analysis with CEL I
endonuclease for all 54 coding exons of NPHP6/CEP290, we
found 59 aberrant banding patterns. Direct sequencing of these
mismatches revealed 15 different nucleotide changes in 7
families with JBTS, 2 families with SLSN and 1 family with
isolated NPHP (tables 1 and 2, fig 1). These consisted of: (1)
five nonsense mutations (C3811T, C4882T, G5941T, T2249G
and C5932T) resulting in premature protein truncation
(R1271X, Q1628X, E1981X, L750X and R1978X); (2) four
deletions (5734delT, 5163delT, 1419-1423delAATAA and 164-
167delCTCA) generating frameshifts and premature stop
T55fsX57); (3) one start codon mutation (A1G); and (4) two
obligatory splice-site mutations (1066-1GRA, 3104-2ARG)
(table 1). Seven of these sequence variations are novel, whereas
C3811T (R1271X)23and 5734delT (W1912fsX1923)23in A372,
and G5941T
5163delT (T1721fsX1723)23 26in F101, T2249G (L750X)25in
F122, and A1991G (D664G)23have already been described. We
did not detect the intron 27 mutation25in our cohort.
In four families (A372, A989, F101 and F57) both mutations
were found in NPHP6/CEP290, thereby indicating the phenotype
to be autosomal recessive (table 1). In four patients (F122,
A1332, F938 and F848), only single heterozygous loss-of-
function mutations could be found (table 1). Individual F848 is
known to harbour a heterozygous missense mutation in NPHP4
When parental DNA was available,
segregation analysis was performed and confirmed that the
sequence variants were transmitted as autosomal recessive
alleles. All the detected sequence variants were absent from 188
control chromosomes of people of Central European, Middle
Eastern, East Asian and American origin.
We also identified several sequence variants of unknown
significance, present in the heterozygous state: one known
missense mutation (A1991G)23and a 3 bp in-frame deletion
(7311–7313delGAA) (fig 1, table 2). The first sequence change
results in a non-conservative amino acid substitution (D664G)
and the conserved residue of the protein (K2437 is conserved in
Ciona intestinalis). However, as this family (A854) was con-
sanguineous, the significance of this change is unknown.
K473fsX478 and
Eight compound heterozygous loss-of-function NPHP6/
CEP290 mutations in four families with JBTS or SLSN
The members of the three families with JBTS (A372, A989 and
F101) and one family with SLSN (F57) in whom eight
found (table 1) exhibited the following disease phenotypes.
658Helou, Otto, Attanasio, et al
Page 3
Table 1
Twelve different truncating NPHP6/CEP290 mutations found in 6 families with JBTS and 2 families with SLSN
of origin
Nucleotide alteration(s)*
Alteration(s) in
coding sequence
Exon (segregation)**
Age at
Ocular features
(age of
diagnosis, years)
Central nervous
system features
32 (het, P)
43 (het, M)
38 (het, ND)
CVA, MR, spastic paresis,
liver fibrosis
44 (het, ND)
splice site
13 (het, P)
LCA (5 months)
MR, empty sella, hypoplasia
of the optic chiasm,
hypothalamic hypoplasia,
(breathing, abnormality,
pes planus)
39 (het, Pwt,M ND)
Empty sella
A1G, 1419-1423del AATAA
Start codon defect
2 (start codon) (het, ND)
NY, early-onset TRD
keratoconus, vision ,1
(early childhood)
Complex focal seizures
(hyperlipidaemia, scoliosis)
15 (het, ND)
23 (het, M)
164_167del CTCA, ?
T55fsX57, ?
3 (het, ND)
None at 1.5
Splice site, ?
29 (het, M)
NY, esotropia, retinal
dystrophy (14)
MR, hypotonia (scoliosis)
44 (het, ND)
TRD (vision 1/10 at 44)
?, Second mutation not found; AT, ataxia; CA, congenital amaurosis (bilateral); CVA, cerebellar vermis aplasia/hypoplasia; ESRD, end-stage renal disease; het, heterozygous in affected individual; hom, homozygous in affected
individual; LCA, Leber congenital amaurosis (blindness within the first 2 years of life); JBTS, diagnosed with Joubert syndrome; M, mutation identified in mother; MEC, occipital menigoencephalocele; MR, mental retardation/psychomotor
retardation; Mwtmaternal sequence is wild type; ND, no data or DNA available; NY, nystagmus; P, mutation identified in father; Pwtpaternal sequence is wild type; SLSN, diagnosed with Senior–Løken syndrome; RC, retinal coloboma;
TRD, tapetoretinal degeneration.
*All mutations were absent from at least 188 chromosomes of healthy controls.
`All patients had renal ultrasonography results compatible with nephronophthisis (increased echogenicity and/or corticomedullary cysts).
1Renal function significantly reduced.
?This patient is also known to have a heterozygous missense mutation in the NPHP4 gene (C1880T, T627M).
**Previously published.
23 25 26
Mutational analysis in NPHP6/CEP290659
Page 4
(1) Two siblings (II-1) and (II-2) from family A372 were
diagnosed with JBTS, LCA and retinal colobomas.
(2) In patient II-1 from family A989, ESRD secondary to
NPHP occurred at 5 years of age. This patient had retinal
dystrophy type LCA, nystagmus, severe mental/psychomotor
retardation, spastic quadraparesis and liver fibrosis. The MRI
showed cerebellar vermis aplasia, supratentorial hypomyelini-
sation and hypoplastic brain stem.
(3) In patient II-2 from family F101, ESRD secondary to NPHP
was diagnosed at 9 years of age, and he was diagnosed with LCA.
Despite the fact that some of the symptoms presented by this
patient are common to JBTS (severe hypotonia in the first year of
life, mental and psychomotor retardation, sleep apnoea episodes),
the CT scan lacked the typical MTS and showed empty sella,
hypoplasia of the optic chiasm and hypothalamic hypoplasia. An
MRI was not available for review.
(4) Patient II-1 from family F57 has been visually impaired
since early childhood, lost vision progressively and was
diagnosed with retinitis pigmentosa at the age of 34 years. He
also had keratoconus. Owing to progressive renal failure
secondary to NPHP, he underwent kidney transplantation at
24 years. He had complex focal seizures at 17 years.
Figure 1
were found in three families with JBTS and one family with SLSN; (B) four single heterozygous NPHP6/CEP290 mutations were found in three families with
JBTS and one family with SLSN; (C) two heterozygous sequence variants of unknown significance were found: one missense mutation in a patient with JBTS
and a 3 bp in-frame deletion in a patient with isolated NPHP (see table 2). Family number, altered nucleotide and amino acid change are given above
sequence traces and wild-type sequence below mutated sequence. One codon triplet in each chromatogram is underlined to indicate reading frame.
Mutations and sequence variants found in NPHP6/CEP290. (A) Eight different compound heterozygous truncating mutations in NPHP6/CEP290
660Helou, Otto, Attanasio, et al
Page 5
Four single heterozygous NPHP6/CEP290 loss-of-
function mutations in four families with JBTS and SLSN
In three patients from families with JBTS (F122, A1332, F938)
and one patient from a family with SLSN (F848), single
heterozygous mutations were found, three of them truncating
(in F122, A1332 and F848) and one splice-site mutation (in
F938) (table 1).
Patient II-2 from family F122 had LCA and features of JBTS,
such as cerebellar vermis aplasia, muscle hypotonia at birth and
later mental/motor retardation. Symptoms and typical ultra-
sound findings of NPHP had been present since 6 years of age.
A1332 presented with dilated calyces bilaterally on ultra-
sound and normal renal function by blood testing at 18 months
of age. He had congenital amaurosis (flat electroretinogram at
15 months of age), nystagmus since birth, cerebellar vermis
aplasia and MTS, and mental and psychomotor retardation. As
his parents are consanguineous but he carries a heterozygous
mutation in NPHP6/CEP290, it is likely that there may be
recessive mutations present in other NPHP genes.
In patient II-1 from family F938, ESRD secondary to NPHP
occurred at 13 years of age. During birth, asphyxia occurred
with hypotonia. In addition, horizontal nystagmus and
esotropia were seen. In early childhood, this patient presented
with severe scoliosis and psychomotor retardation. An ophthal-
mological examination revealed retinal dystrophy.
In patient II-1 from family F848, initial symptoms of NPHP
occurred during infancy. However, ESRD occurred late, at the
age of 40 years. The patient progressively lost vision and at
44 years of age, retinitis pigmentosa (SLSN) was diagnosed. He
also had bilateral cataracts. This patient is known to harbour a
heterozygous missense mutation in the NPHP4 gene, which has
been published previously.28
Phenotypes of families with sequence variants of
unknown significance
In patient II-1 from family F459, a nonconservative missense
mutation, A1991G (D664G), was found. In a recessive disease,
this sequence variant alone cannot be disease causing. The
patient had cerebellar vermis hypoplasia, ataxia, developmental
delay and progressive hepatic fibrosis; the latter has previously
been described in COACH syndrome.15In addition, the patient
presented with hearing loss, which has not been described
(table 2).
As oligogenic mutations of the NPHP1, AHI1 and NPHP6
genes have been described in JBTS, we examined the patients
who carried two mutations or one single heterozygous
mutation in NPHP6 for additional mutations in NPHP1 and
AHI1. However, we did not detect any additional mutations in
either gene.
NPHP6/CEP290 mutations in isolated NPHP
Interestingly, in a patient with isolated NPHP (patient II-9 from
family A854) a 3 bp in-frame deletion (7311–7313delGAA;
K2437del) was found (table 2), which was absent in 188
healthy control chromosomes. It deletes an amino acid residue
conserved in the C intestinalis orthologue of NPHP6/CEP290.
Renal symptoms (polyuria, polydipsia) and increased echo-
genicity on ultrasonography occurred at 10 years of age. To test
the possibility of NPHP6/CEP290 mutations in isolated NPHP,
we analysed 5 families that had been found to be homozygous
at the NPHP6 locus when we had performed a total genome
search for homozygosity in 105 families, and found these 5
families to be homozygous for markers in the NPHP6/CEP290
gene region. We did not find any sequence variants in these
In this study, we performed mutational analysis in 195 families
with JBTS, SLSN or isolated NPHP. In total, 15 different
nucleotide changes were found, 7 of them novel. In four
patients, both compound heterozygous truncating or splice site
mutations were found. In another four patients, only single
heterozygous mutations were found; three of these mutations
were truncating and one was a splice defect (table 1). The single
heterozygous mutations are not disease-causing in themselves
in the recessive disease NPHP, but could be disease-causing in
combination with mutations in other genes.26 31
We report here one family (F848) with a heterozygous
nonsense mutation in NPHP6/CEP290 and an additional known
missense mutation in NPHP4, as published previously.28In one
consanguineous family (A1332) only a single heterozygous
truncating mutation could be found. Tory et al reported a
patient with a homozygous deletion in NPHP1 and a hetero-
zygous truncating mutation in NPHP6/CEP290.24These muta-
tions may potentially indicate a situation of oligogenic
inheritance as described in patients with Bardet–Biedl syn-
In the mouse model of retinal degeneration, rd16, a 300
amino acid in-frame deletion as a hypomorphic allele was
found to be associated with early-onset retinal degeneration.27
This is consistent with the finding that the hypomorphic allele
of a partial splice defect in intron 2725in humans leads to an
ocular phenotype only, and not to renal or cerebellar involve-
ment. Interestingly, neither kidney nor gross brain pathological
changes could be found in the rd16 mice. Mutations in NPHP6/
CEP290 have been described as the most common genetic cause
Table 2
NPHP6/CEP290 sequence variants of unknown significance
Alteration(s) in
coding sequence?
Age at
(age of
Central nervous
system features (other)
D664G, ? (NC)21 (het,Mwt)–NDJBTS NDCVA, AT, MR, RC
(hepatic fibrosis,
hearing loss)
None A854 (II-9) Pakistan 7311-7313delGAA, ?
K2437del (C. i.), ?
55 (het, ND)
?, Second mutation not found; AT, ataxia; CVA, cerebellar vermis aplasia/hypoplasia; ESRD, end-stage renal disease; het, heterozygous in affected individual; JBTS,
diagnosed with Joubert syndrome; M, mutation identified in mother; MR, mental retardation/psychomotor retardation; Mwtmaternal sequence is wild type; ND, no data
or DNA available; P, mutation identified in father; PC, parental consan-guinity; SLSN, diagnosed with Senior–Løken syndrome; RC, retinal coloboma.
*All mutations were absent from at least 188 chromosomes of healthy controls.
?Evolutionary conservation: NC, non-conserved; C. i., Ciona intestinalis.
`All patients had renal ultrasonography results compatible with nephronophthisis (increased echogenicity and/or corticomedullary cysts).
`Previously published.
Mutational analysis in NPHP6/CEP290 661
Page 6
of LCA/early onset retinal degeneration.25In our cohort there
were 11 patients with diagnosed LCA. Interestingly, mutations
in NPHP6/CEP290 were found in five of these patients. All of the
patients with JBTS with either compound or single hetero-
zygous NPHP6/CEP290 mutations identified in this study
presented with an ocular phenotype, confirming that this gene
is a major cause of JBTS with oculorenal involvement as
described previously.23
NPHP6/CEP290 mutations can be found in patients with a
spectrum of phenotypes, mainly JBTS or early-onset retinal
degeneration with additional extrarenal manifestations. We
also found NPHP6/CEP290 mutations in a patient with empty-
sella syndrome and in a patient with JBTS and an additional
manifestation of liver fibrosis. In summary, we have found six
novel loss-of-function mutations of NPHP6/CEP290.
We express our sincere appreciation to the patients and their families
for their participation in this study. We gratefully acknowledge the
cooperation of the following colleagues: A Kampik and G Rudolph (Eye
Hospital, LMU Mu ¨nchen, Germany); R Weleber (Casey Eye Institute,
Oregon Health and Sciences University, USA); R Beetz (Children's
Hospital, Johannes-Gutenberg Universita ¨t Mainz, Germany); L
Schimmenti, (Pediatrics, UCLA, USA); Y Pirson (Universite? Catholic
de Louvain, Brussels, Belgium); D Walb (Wiesbaden, Germany); T J
Neuhaus and G Laube (Children's Hospital, Zurich, Switzerland); and S
Signori (Department of Child Neurology and Psychiatry, IRCCS ''C
Mondino'' Foundation, University of Pavia, Italy). H Omran is
supported by grant no. SFB592; FH is a Frederick G L Huetwell
professor and Doris Duke Distinguished Clinical Scientist, and is
supported by grants from the NIH (DK068306, DK064614 and
Authors' affiliations
Juliana Helou, Edgar A Otto, Massimo Attanasio, Susan J Allen, John
F O'Toole, John A Sayer, Friedhelm Hildebrandt, Departments of
Pediatrics and of Human Genetics, University of Michigan, Ann Arbor,
Michigan, USA
Melissa A Parisi, Ian Glass, Department of Pediatrics, University of
Washington, Seattle, Washington, USA
Boris Utsch, Department of Pediatrics, University of Erlangen, Erlangen,
Seema Hashmi, Department of Pediatric Nephrology, Sindh Institute of
Urology and Transplantation, Karachi, Pakistan
Elisa Fazzi, Department of Child Neurology and Psychiatry, University of
Pavia, IRCCS ''C. Mondino'' Foundation Italy
Heymut Omran, Department of Pediatrics, University of Freiburg, Freiburg,
John A Sayer, Department of Human Genetics, School of Clinical Medical
Sciences, University of Newcastle upon Tyne NE1 3BZ, UK
Competing interests: None declared.
Correspondence to: Friedhelm Hildebrandt, MD, University of Michigan
Medical School, 8220C MSRB III, 1150 West Medical Center Drive, Ann
Arbor, MI 48109-0646, USA; email@example.com
Received 27 May 2007
Revised 19 June 2007
Accepted 20 June 2007
Published Online First 6 July 2007
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Key points
N Mutations in the gene NPHP6/CEP290, which encodes
the centrosomal protein nephrocystin-6, cause nephro-
nophthisis-associated ciliopathies.
N In this study, we examined a worldwide cohort of 195
families with Senior–Løken syndrome (SLSN), Joubert
syndrome (JBTS) and isolated nephronophthisis for
mutations in NPHP.
N We identified seven novel and seven known mutations in
seven families with JBTS, two families with SLSN and one
family with isolated NPHP, thus confirming the clinical
heterogeneity of patients with NPHP6/CEP290 muta-
662Helou, Otto, Attanasio, et al
Page 7
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Used three times while waiting on a bigger bunn coffee maker to be shipped call 803-524-7840
washer and dryer
I have an Amana washer and a GE dryer it came in the house we bought it is used we checked them both out before unhooking them. They are in good condition. Must pick up yourself.
Mar 16 -
We are a dealer of used appliances that sells exclusively to stores and exporters throughout the U.S and overseas.
For the past 25 years we have worked hard to ENSURE that our clientele receive the affordable and reliable, appliances THEY NEED TO, gain an edge over the ...
Mar 13 -
Chef Mate Microwave Preowned
This is a Chefmate Microwave that is preowned
Great for apartment or college dorm
Mar 11 -
White Kenmore Washing Machine
White Kenmore Washer, believe it is a 15 drum size, used once, looks like new. Bought in Jan.2014, so I would say it's a little over a year old. House I was living in caught fire
and I bought machine when I moved, but was also given another machine, which is larger. If ...
Mar 10 -
Waring 7 Speed Blender
Like new in original box with instructions, instant on/off pulse blend action for blending control, made to last
Mar 8 -
Mixing Bowls, Thermal Glove & Cake decorating kit
A set of three plastic mixing bowls, a thermal glove & a cake decorating kit, all for one low price!
Bud Light mini fridge
Like new Bud Light mini fridge!
Mar 7 -
New GE COOK TOP GAS
GE STAINLESS STEEL GAS COOK TOP JGP633SETSS 5 BURNER.NO BOX NEVER USED.contact Wendi 7049381516 RETAILS AT LOCAL HARDWARE STORE FOR 800.00.NO REASONABLE OFFER WILL BE REFUSED.
Mar 3 -
Samsung Wah Machine and Dryer Pair Like New!
We purchased these when we moved into our apartment. We are building a house and will be getting a new pair. We have only have them 6 months. Awesome washer and dryer for an awesome deal! They have a ten year manufacturers warranty as well.
Feb 26 -
General Electric 16 cu ft chest freezer
1 year old chest freezer. moving must sell.
Feb 24 -
KitchenAid Roto Slicer, Shredder and Food Grinder
Brand new with original box, never been used.
Roto Slicer and Shredder
- includes 2 slicers, makes thick or thin slices of firm fruits and vegetables.
- includes 2 shredders, makes fine or coarse nuts, dried fruit, firm cheese and vegetables.
Food Grinder
- includes fine ...
Feb 24 -
1950's Frigidaire Refrigerator Deluxe
1950's Frigidaire Deluxe Refrigerator. Ice cold. Dimensions: 24" wide; 5'4" tall; 32" width. One door with upper freezer compartment. White outside, pink inside
Feb 20 -


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Kristine Lane
Haverhill, Massachusetts, 01832
Commute to Downtown Haverhill
Almost all errands require a car.
Score Details
Kristine Lane has a Walk Score of 1 out of 100. This location is a Car-Dependent neighborhood so almost all errands require a car.
This location is in Haverhill.
The closest school is Hilltop School.
Nearby restaurants include The Grill Next Door, Brewer's Market and One Stop Market.
Kristine Lane is near American Legion Field/Clement Farm, Consentino Field and Cashmans Field.
Explore how far you can travel by car, bus, bike and foot from Kristine Lane.
View all Haverhill apartments on a map.
Popular apartment searches include hardwood floor, fireplace and pool. View all Haverhill apartments and rentals.
This location is in the city of Haverhill, MA. Haverhill has an average Walk Score of 39 and has 60,879 residents.Learn More About Haverhill


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Building a home in WA in June - Layout & Elevation
tlpettyApril 13, 2014
So, we are building a bit of a unique home near a lake in northern Washington this coming June. I will post the plans and elevation here for thoughts. I might need to do multiple posts for my million questions...but, lets start with the plans and elevation! We are on a budget, so we are very concerned with saving money while still getting our big "wants" and "needs", so layout is HUGE. The right "wing" of the house to the right of the exercise room is an in-laws suite and the left wing is our home. Give me your thought and tell me what issues in any capacity you see that I might need to think over, please!?
We also changed the window design to this:


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What we do:
We offer custom built SUP's (Stand Up Paddle Boards) , Surfboards, Skateboards and Snowboards. Our custom SUP's and Surfboards are shaped and built by Champion Surfer Mike Estrada "Estrats"in Newport Beach, CA in the good old USA. Our surf inspired "Complete Skateboards" are designed, hand shaped and CNC cut in Sarasota, FL and Carlsbad, CA. They can be custom configured with premium quality components including color pinstripes. Our new "All Mountain Snowboards" are made in the USA as well with quality craftsmanship and superior performance being the key ingrediants. Our quality apparel in a variety of technical and cotton t-shirts and hats are all offered at affordable prices. We believe in keeping it "local" yet affordable whenever possible.
We are enjoying the winter but are also revving up for Spring!
Winter is still here and the crew has been boarding as much as we can! Snow has been fun and if you have not snowshoe hiked, be sure you try it! Super fun and great workout! Check out our new Snowboard (da Mountain Goat) for next season as well! Super stoked on these boards!
We are anxious to see what you all have been up to? Hit us up on our FishPlanx Facebook site! We want to see you tearing it up either in the water, parks, streets and even the snow! We will have a page where our friends can post up their pics either sporting FishPlanx clothing or boarding on one of our boards or both! Look for this soon and looking forward to seeing the pics as well!
The FishPlanx Crew!
Follow us on:


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One of San Francisco's most festive holiday events will brighten up the Castro district on Sunday, December 16. Men and women from all walks of life will don boxers, briefs, and bras to raise money for San Francisco AIDS Foundation in the fourth annual Santa Skivvies Run. We sat down with event director Robin Easterbrook to find out what makes this event so special, and what's new this year!
For people who've never gone to the Santa Skivvies Run, describe it for us?
It truly is an only-in-San Francisco kind of event. It's an amazing collection of people from all walks of life who gather together to run through the streets of the Castro neighborhood in their skivvies to support a very worthy cause. For those who are a little more modest, underwear is not required—we have a lot of people who dress up as elves, Santas, reindeer, or whatever costume they chose. The word that best describes the event for me is "joy." Everyone is just so happy and joyful. They're thrilled to be a part of this amazing event!
Not only is this a fun event, it has a purpose.
Yes, it does. The event supports the foundation's free, local HIV prevention and care services that benefit thousands of clients annually, especially in hard-hit neighborhoods like the Castro. We rely on events like the Santa Skivvies run to keep our programs strong and stable so that people can rely on San Francisco AIDS Foundation to be there when they need it.
Tell us about the route.
The run begins and ends at Lookout (3600 16th Street), which is the official host of the Santa Skivvies Run for the fourth consecutive year! (We love Lookout!) The route winds its way through the Castro. This year, we will have drag queens leading the way on Segway scooters. I can't wait to see that!
And you've added something new this year?
Yes, it's very exciting. This year we will be blocking off Noe Street, between Market and Beaver Streets, to create our first-ever Santa Skivvies Village. It will be set up right outside Lookout and will include a stage for performances by our drag hosts as well as a few surprises for the runners.
Tell us about fundraising. It's easy, right?
We make it very easy for participants to raise funds to support the foundation. Fundraising for the Santa Skivvies Run is optional, but participants are encouraged to create a fundraising goal. We provide online tools and great incentives to help each participant meet and exceed their goal. Last year our dedicated runners raised more than $30,000.
How much does it cost to participate?
Registration is $35, and all runners receive a Santa hat as a registration gift. Participants can register the day of the event, from 10:00 a.m. to 12:30 p.m. at Lookout.
It sounds like you have one of the best jobs in the world…
I love my job. I mean, I get to see people running around in their underwear! More than anything, it's just so rewarding to see people coming out in such a bold way to support San Francisco AIDS Foundation. We have the most loyal supporters in the world and I'm always so proud of all of our participants. I would encourage anyone thinking about taking part in the Santa Skivvies Run to join us. You'll have a blast!
To register or learn about volunteering opportunities, visit www.santaskivviesrun.org.
To see more photos from last year, check out Santa Skivvies Run on Facebook.
News and discussion of HIV/AIDS treatment, prevention and policy on our Facebook page. Join us!
Positive Force program manager T.J. Lee ensures clients get the support, information and services they need. ...more
Bridgemen brings gay, bi and trans men together for community service and social events. Find out from Jared Hemming what the group is all about. ...more
Tim Patriarca, answers our questions about the new gay and bi men's center for health and wellness opening this spring. ...more
Rick supports speed users who want to change or reduce their drug use in the PROP program. Read about how his unique expertise in holistic health care and harm reduction helps clients. ...more
Look out for our new testing RV now bringing testing services to a neighborhood near you. ...more


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Encountering Race Through Poetry – Paula Cole Jones speaks with a poet's heart, with a longing to pen words that resonate in the lives of others.
Progressive Media Advocates
Carole Bartolotto: The problem with concluding that GMOs are safe is that the argument for their safety rests solely on animal studies. These studies are offered as evidence that the debate over GMOs is over. Nothing could be further from the truth.
Steve Hochstadt: The Republicans in Congress, virtually all of whom publically deny that climate change is occurring, have just proposed a budget which cuts or reverses all the programs which might reduce the pace of warming.
Joe Mathews: Thanks to Xi Jinping, Chinese millionaires have propped up the state's sagging real estate market, universities, and companies.