Clues to cell death in ALS

Aggregations of misfolded proteins foretell cell death in ALS model

Susan Brown(meldahlbrown@hotmail.com)
Oct 31, 2005

Neuronal cells clogged with a mutant protein associated with amyotrophic lateral sclerosis (ALS) die within hours after the clumps first form, researchers report in this month's Journal of Cell Biology. The finding directly links aggregation of malformed proteins with cell death characteristic of the disease, the authors claim.

"This is the first study that has shown reasonable evidence that aggregates are involved in neurotoxicity," said neurologist Michael Weiss of the University of Washington in Seattle, who conducts clinical research on ALS but was not involved in this project.

Motor neurons in the brain and spinal cord decay in patients with the illness, also known as Lou Gehrig's disease. Without those neurons, muscles wither, leading to death within a few years. About 20 percent of ALS patients with the familial form of the disease have inherited a mutant form of the gene for superoxide dismutase (SOD1), which causes the protein to...

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