Article
Restricted expression of mutant SOD1 in spinal motor neurons and interneurons induces motor neuron pathology.
Neurobiology of disease - 1 Mar 2008
Wang Lijun, Sharma Kamal, Deng Han-Xiang, Siddique Teepu, Grisotti Gabriella, Liu Erdong, Roos Raymond P
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the selective loss of motor neurons (MNs). Approximately 10% of ALS cases are familial (known as FALS), and approximately 20% of FALS cases are caused by mutations in Cu/Zn superoxide dismutase type 1 (SOD1). Mutant (MT) SOD1 induces FALS as a result of a toxicity that remains poorly defined. Several studies suggest that the...
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