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Hutchinson-Gilford syndrome

Progeria is an extremely rare genetic condition which causes physical changes that resemble greatly accelerated aging in sufferers. The disease affects around 100 in 48 million newborns. Currently, there are approximately 35 known cases in the world. There is no known cure. Most people with progeria die around 13 years of age. Progeria is of interest to scientists because the disease may reveal clues about factors involved in the process of aging, because it is an "accelerated aging" disease. But unlike most other "accelerated aging diseases" (like Werner's syndrome), progeria is not caused by defective DNA repair. more...

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The condition was first identified in 1886 by Jonathan Hutchinson and Hastings Gilford. The condition was later named Hutchinson-Gilford Progeria syndrome; the name was derived from the Greek for "prematurely old". Around 100 cases have been definitely identified since then.

Cause

According to 'recent evidence, Progeria may be a de novo dominant trait. It develops during cell division in a newly conceived child or in the gametes of one of the parents. It is caused by mutations in a LMNA (Lamin A' protein) gene on chromosome 1.

Symptoms

Symptoms generally begin appearing around 18-24 months of age. The condition is distinguished by limited growth, alopecia and a characteristic appearance with small face and jaw and pinched nose. Later the condition causes wrinkled skin, atherosclerosis and cardiovascular problems. Mental development is affected. Individuals with the condition rarely live more than 16 years; the longest recorded life-span was 26 years. The development of symptoms is comparable to aging at a rate six to eight times faster than normal, although certain age-related conditions do not occur.

Read more at Wikipedia.org


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Chip Foose: humble genius
From Automotive Design & Production, 4/1/05 by Christopher A. Sawyer

SCENE 1

After speaking at Detroit's Cobo Hall during the North American International Auto Show, Chip Foose seems genuinely taken with the evident adulation of the audience, and takes the time to answer every question and sign autographs.

[ILLUSTRATION OMITTED]

The second oldest child and only male in a family with four kids, Chip Foose was born in Santa Barbara, California, on October 6, 1963. It's doubtful he could have imagined he'd have a television show (Overhaulin'), a successful business creating custom-made vehicles (Foose Design), that he'd graduate from the Art Center College of Design, or be a design consultant to the Big Three. Then again, he might. Not only does his mind spill over with ideas, his wasn't a normal childhood.

"I spent the weekends of the first three years of my life at [model car maker] AMT with my dad, who was building their show cars along with Gene Winfield," Foose recalls. When Sam Foose moved over to Minicars to build government-funded safety car prototypes, Chip tagged along. At the age of 7, he joined his dad's company, Project Design, and learned to do body and paint work. "Dad likes to say I worked my way up to my allowance of 17 cents an hour in seven years," he deadpans.

Foose learned basic drawing skills by copying his dad's technique and designs, and progressed to drawing the vehicles the shop would build. There he met former Ford and Tucker designer Alex Tremulis, and also took over building scale models for Tremulis from his father. Impressed, Tremulis told Chip about Art Center, and suggested he attend. Unfortunately, it proved to be more expensive than living in the converted tool shed at his parent's house ("I loved that," he says), and he was forced to leave two years into his studies. To make ends meet, he did illustrations for magazines, worked with his father, and freelanced for Stehrenberger-Clenet Design as it morphed into ASHA Corp. Moderately successful, he felt no need to continue his studies.

SCENE 2

While sitting in the second floor lounge of BMW's show floor stand, fellow speech-maker and designer Chris Bangle, BMW's chief of Design, points to Foose and announces for all to hear: "This is one of the most important and influential automotive designers in the world today." It's high praise from a man about whom the same can be said.

At ASHA he learned to build full-size clay models, pull molds, build bodies ... and draw. "When I left Art Center, I really didn't know how to draw what I was thinking," he says with no sense of irony, "but the four years I spent as Mark Stehrenberger's assistant showed me how to stop fighting the process." Around this time, his youngest sister, Amy, died of Hutchinson-Gilford Progeria Syndrome, a disease that prematurely ages its victim and leads to an early death. (To read about Amy and her disease, visit www.progeriaresearch.org) Devastated by the loss, Chip adopted her can-do, optimistic attitude in tribute. It serves him well.

He returned to school with AHSA backing when his newly minted lawyer girlfriend announced she wouldn't marry him unless he completed his degree. Others, however, wish he had stayed away. "His modeling and drawing skills made it damned tough on those of us who followed him," says BMW's Bangle with a smile. "He raised them to an art form ... We truly hated him." Lynne, however, didn't. She became his wife and business partner.

Foose bought his way out of his ASHA contract, interviewed with both VW and Ford, and accepted Ford's offer to come to Dearborn. He'd been working for hot rodder Boyd Coddington on the side for two years, "building cars and having a ball," when Boyd suddenly trumped Ford's offer to keep him from leaving. "I never thought that what I loved to do--designing and building hot rods and show cars--could ever be a career," he says, "but I figured I was still young enough that I could join a carmaker if it didn't pan out. Even if the thought of designing door handles for the rest of my life didn't excite me ..."

[ILLUSTRATION OMITTED]

Years later, as Hot Rods by Boyd descended into bankruptcy. Foose was faced with a choice: He could continue to buy parts for client's cars with his own money, or strike out on his own. On the day he left, he discovered Lynne was pregnant with their first child, his mortgage payment was due, and he had just $700 in the bank. Drawing from the example set by his late sister, he refused to lose hope. One week later, mobile audio company Precision Power called with a $10,000 advance, and Foose Design was in business designing its next set of car audio components.

Because of this experience, Foose prefers to keep things manageable. His company occupies a miniscule 5,000-[ft.sup.2] building in Huntington Beach, CA, and subcontracts work to other builders-among them hot rod legends Troy Trepanier, Alan Johnson, and Steve Moal. Often his name is not attached to the project. "Let someone else build their company a little," he says. "I'm just the designer."

[ILLUSTRATION OMITTED]

SCENE 3

Kip Wasenko, father of Cadillac's new design language, shows Foose around the V-Series cars on the Cadillac stand. A crowd of 60 stand and watch in near-reverence. Foose draws on his hands-on experience to show Wasenko how changes to the STS-V's front seats will bring their look and feel into line with what his team originally wanted. Wasenko is pleased.

Foose says design students and professionals alike should spend less time on the computer, and more time sketching and building models. "If you can't draw it and you can't build it, that's not design--it's an idea," he observes. "You don't begin to design until you know how to communicate those ideas into two or three dimensions." He admits he's intrigued by the idea of rendering a vehicle on a computer but confesses, "I just don't have the time to learn it." "Besides," he says, "sketches are much more mobile.

[ILLUSTRATION OMITTED]

I can go anywhere and communicate my ideas with a sketch."

Increasingly, those places include car company design departments. A firm believer that a passionate design emotionally attaches a buyer to a vehicle, Foose says assimilating lines and gestures from past vehicles--where passion was the main focus--to create new designs doesn't have to result in a vehicle that looks backward. "It's no different than what GM did when designing the Corvette," he says. "If you know where to look, you can see lines and gestures from past Corvettes, things that really tug at the buyer's emotions and make the sale." When asked about his contributions to Detroit's recent design heritage, Foose resolutely refuses to identify the concept or production vehicles on which he's consulted. Yet many in the industry say Ford's Forty Nine, Mustang, and Sport Trac concepts carry his signature style. "J Mays is a good friend, and has been very nice to me," is all Foose says when asked.

SCENE 4

Back in the ballroom where Foose spoke four hours earlier, John Lasseter, the writer, director and producer of Pixar's animated film Cars, says "hello." Foose worked on the vehicles in the movie and created the ever-changing tattoo paint job on the 1959 Chevy character named "Ramon."

Despite a schedule that might kill other men, Foose hopes to expand into an exclusive arrangement with an automaker to produce signature vehicles. These would include wheel, tire, interior and exterior treatments at the base, and expand to encompass unique vehicles at the top. "It could be anything from a sports car to a minivan," says Foose, "and combine the aftermarket and OEM sides. It doesn't even have to have my name on it. I just want to get my ideas out there." It's then, with the almost off-hand way he says it that you realize the humility, like his design ability, is real.

By Christopher A. Sawyer, Executive Editor

COPYRIGHT 2005 Gardner Publications, Inc.
COPYRIGHT 2005 Gale Group

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