Article
Lysyl-tRNA synthetase is a target for mutant SOD1 toxicity in mitochondria.
The Journal of biological chemistry - 17 Oct 2008
Kawamata Hibiki, Magrané Jordi, Kunst Catherine, King Michael P, Manfredi Giovanni
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease affecting the motor neurons. The majority of familial forms of ALS are caused by mutations in the Cu,Zn-superoxide dismutase (SOD1). In mutant SOD1 spinal cord motor neurons, mitochondria develop abnormal morphology, bioenergetic defects, and degeneration. However, the mechanisms of mitochondrial toxicity are still unclear. One...
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