Article
The legs at odd angles (Loa) mutation in cytoplasmic dynein ameliorates mitochondrial function in SOD1G93A mouse model for motor neuron disease.
The Journal of biological chemistry - 11 Jun 2010
El-Kadi Ali Morsi, Bros-Facer Virginie, Deng Wenhan, Philpott Amelia, Stoddart Eleanor, Banks Gareth, Jackson Graham S, Fisher Elizabeth M C, Duchen Michael R, Greensmith Linda, Moore Anthony L, Hafezparast Majid
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a debilitating and fatal late-onset neurodegenerative disease. Familial cases of ALS (FALS) constitute approximately 10% of all ALS cases, and mutant superoxide dismutase 1 (SOD1) is found in 15-20% of FALS. SOD1 mutations confer a toxic gain of unknown function to the protein that specifically targets the motor neurons in the cortex and the spinal cord. We have previously...
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