Copyright 2004 U.P.I.  
United Press International
April 28, 2004 Wednesday 13:19 PM Eastern Time
LENGTH: 207 words
DATELINE: TUCSON, April 28 (UPI)

A gene for a rare juvenile-onset form of Lou Gehrig's disease has been isolated in U.S., Belgian and Austrian families.

The newly discovered gene carries instructions for a protein called senataxin, the Muscular Dystrophy Association said of the finding on amyotrophic lateral sclerosis, as the disease is formally known.

Senataxin helps cells interpret and process genetic instructions along the biochemical pathway from DNA to protein manufacturing.

When senataxin is flawed, as it is in ALS-affected families, this pathway is apparently disrupted, leading to the gradual death of muscle-controlling nerve cells in the brain and spinal cord.

"This discovery fills in one more piece in the puzzle of the causes of ALS," said neurologist Valerie Cwik, the association's medical director.

"It also provides a new line of investigation for the development of treatments for this devastating disease."

The senataxin finding, though it explains only a small percentage of ALS cases, may have implications for the larger world of ALS.

Most cases of ALS aren't clearly genetic, begin in late middle age, and progress rapidly to paralysis and death.

Researchers at the University of Washington in Seattle conducted the research.