| Packard Center for ALS Research at Johns Hopkins |
Aug. 27, 2010 — ScienceDaily Neuron-Damaging Mechanism Discovered in Mouse Model of Inherited ALSNew research uncovers what may be a primary neuron-damaging insult that occurs in an inherited form of a devastating neurodegenerative disorder. The study, published in the August 26th issue of the journal Neuron, describes a critical mechanistic link between a mutant protein and disease pathogenesis in an animal model of amyotrophic lateral sclerosis (ALS). ALS is a disease that attacks the neurons in the brain and spinal cord that control voluntary movement. There is no cure for ALS, which typically develops in adults and is characterized by a progressive paralysis that often leads to death within three to five years of diagnosis. About 10% of ALS cases are inherited and a portion of those are attributed to mutations in the gene for a protein called SOD1. However, the exact mechanism that links mutant SOD1 with motor neuron degeneration has not been established. | Events
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