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Zellweger syndrome

Zellweger syndrome is a rare, congenital disorder (present at birth), characterized by the reduction or absence of peroxisomes (cell structures that rid the body of toxic substances) in the cells of the liver, kidneys, and brain. It is characterized by an individual's inability to beta-oxidize very-long chain fatty acids in the peroxisomes of the cell, due to a genetic disorder in the PEX2 gene. more...

Zadik Barak Levin syndrome
ZAP70 deficiency
Zellweger syndrome
Zollinger-Ellison syndrome

Named after Hans Zellweger, a former professor of Pediatrics and Genetics at the University of Iowa who did research into the disease, it is also called cerebrohepatorenal syndrome.

VL chain fatty acids are generally found in the central nervous system (brain and spinal cord) and the peroxisomes of these cells cannot import the necessary degrative proteins for B-oxidation to occur. Zellweger syndrome is one of a group of genetic disorders called peroxisomal diseases that affect brain development and the growth of the myelin sheath, the fatty covering—which acts as an insulator—on nerve fibers in the brain.

Symptoms are often exhibited at around 1 to 2 years of age. If left untreated Zellweger's syndrome can lead to major mental retardation and death. The other most common features of Zellweger syndrome include an enlarged liver, high levels of iron and copper in the blood, and vision disturbances. Some affected infants may show prenatal growth failure. Symptoms at birth may include lack of muscle tone and an inability to move. Other symptoms may include unusual facial characteristics, mental retardation, seizures, and an inability to suck and/or swallow. Jaundice and gastrointestinal bleeding may also occur.

There is no cure for Zellweger syndrome, nor is there a standard course of treatment. Infections should be guarded against to prevent such complications as pneumonia and respiratory distress. Other treatment is symptomatic and supportive. The prognosis for individuals with Zellweger syndrome is poor. Death usually occurs within 6 months after onset, and may be caused by respiratory distress, gastrointestinal bleeding, or liver failure.


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Crowe lands knockout with 'Cinderella Man'
From Spokesman Review, The (Spokane), 6/3/05 by Dan Webster Staff writer

Whatever else you can say about it, and you can say plenty, boxing remains the essence of sport.

Football? Sure you carry a ball, but the idea is to knock somebody down so that you can run from here to there. Hockey? Please. Basketball? Have you even seen the Detroit Pistons play?

No, it's not as popular as it once was, but boxing - even more so than amateur wrestling or the various martial arts - pits one athlete against the next, each punching until one is beaten. Sometimes, and often, unconscious.

That's the backdrop against which you have to measure a film such as "Cinderella Man," Ron Howard's biopic of the one-time heavyweight champion of the world, James J. Braddock.

The other consideration, of course, is the time in which Braddock fought - 1926 to 1937. And that period, which includes the 1929 stock market crash and the Great Depression, is important because it's what Howard (with the help of screenwriters Alvin Hollingsworth and Akiva Goldsman) uses to make "Cinderella Man" into a boxing equivalent of "Seabiscuit."

Braddock (played convincingly by Russell Crowe) was a natural light-heavyweight. Standing nearly 6-feet-3 and weighing barely 190 pounds, he was an early success. But injuries, mainly to his hands, coupled with the same kind of severe economic problems facing the nation as a whole, put Braddock on the financial ropes. By the early '30s, he was struggling to find work on the docks by day, fighting - and often losing to pugs - at night.

Then he got his second chance. Upset wins over three highly regarded fighters landed him a 1935 fight with heavyweight champion Max Baer - a guy with a punch so hard that if you took one, as one wag put it, "you flew into the third row." One of Baer's opponents actually died of a concussion.

That's the film that Howard makes: a fighter, dubbed "Cinderella Man" by sportswriter Damon Runyon for his rags-to- riches rise, struggling to support his family by stepping into the ring with a potential killer.

And as the movie would lead you to believe, the crowd - many of whom are as bad off as Braddock - gets solidly behind their working- class hero. "He raised a nation's spirit," reads the film's tagline, "and gave us the strength to keep on fighting."

See? "Seabiscuit," right?

Despite Howard's tendency to play with the truth (Baer was actually a popular guy and not the killer played here by Craig Bierko), "Cinderella Man" is Howard's most straightforward film since "Apollo 13." And showing a kind of humility that must be hard for him, Crowe is a natural as Braddock, both in the ring as a sturdy fighter and out as a loving father.

Other actors are less successful. Renee Zellweger tears up a lot as Braddock's brave wife, Paddy Considine is cast as the obligatory radical-come-to-a-bad-end and Paul Giamatti plays Braddock's manager at times as if he were Harold Hill looking for trombones to lead the big parade.

No matter. While "Cinderella Man" avoids the "Rocky" syndrome (which might not mean what you think), the film does have a feel of authenticity that is built around Crowe's quiet intensity. It feels far shorter than its near-2 ½-hour running time.

And Howard manages to stage convincing boxing scenes that do make the sport appear as brutal as it really is. And always has been.

SIDEBAR: "CINDERELLA MAN" -- -- -- Locations: NorthTown Mall, River Park Square, Spokane Valley Mall, Showboat cinemas Credits: Directed by Ron Howard, starring Russell Crowe, Renee Zellweger, Paul Giamatti, Paddy Considine, Bruce McGill, Craig Bierko Running time: 2:24 Rating: PG-13 (intense boxing violence, language)

Copyright c 2005 The Spokesman-Review
Provided by ProQuest Information and Learning Company. All rights Reserved.

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