Submission + - Gehrig's Discovery Sparks Hope (thestar.com)
Raver32 writes: "Hunched over her microscope at the University of Toronto, Janice Robertson is focused on innocuous-looking brown blobs.
She's been hunting for life-saving clues into the mystery of amyotrophic lateral sclerosis (ALS), the muscle-destroying killer known as Lou Gehrig's disease.
It has perplexed researchers for nearly 140 years and it is a mystery that has captivated Robertson as she watches the microscopic round cells — motor neurons in minuscule sections of human spinal cord and brain.
In ALS, these motor neurons are killed by mutant genes that make defective proteins, she explains, causing paralysis and death usually within five years.
Named for the New York Yankees player killed by the disease in 1941, Lou Gehrig's has also laid waste to physicist Stephen Hawking and claimed the lives of Sesame Street director Jon Stone, jazz legend Charlie Mingus, actor David Niven, composer Dimitri Shostakovich, and Chinese Communist leader Mao Zedong.
Effective treatment and a cure do not exist.
But Robertson and a Toronto team of scientists have developed the world's first antibody to the abnormal protein derived from the mutant superoxide-dimutase-1 (SOD1) gene, the only known cause of Lou Gehrig's, and responsible for 2 per cent of all cases. This antibody could be used to detect and remove the abnormal forms of the protein.
The scientists say their findings, published in the June edition of Nature Medicine, open the door to ways for better treatments, prevention and earlier diagnosis."
