University of Massachusetts Medical Center,
Department of Neurology, Room S5-740
55 Lake Avenue North,
Worcester
MA-01655, USA
Amyotrophic lateral sclerosis(ALS), often referred to as "Lou Gehrig's disease" is an age-dependent degenerative disorder of motor neurons in the spinal cord and brain. Dysfunction of both lower motor neurons(weakness, fasciculations) and upper motor neurons(weakness, hyperreflexia, spasticity) follows a progressive course, leading to death from respiratory paralysis, usually within five years of onset.
Although mutations in the gene encoding Cu, Zn-Superoxice dismutase (SOD1) were identified as a cause of familial ALS(FALS), the mechanism by which they cause age-dependent neurodegeneration remains unclear. Our Goal is to apply biophyiscal and biochemical methods to analyze structural and functional changes caused by mutant residues in purified SOD1 variants.
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