Article
Differential effects of mutant SOD1 on protein structure of skeletal muscle and spinal cord of familial amyotrophic lateral sclerosis: role of chaperone network.
Biochemical and biophysical research communications - 16 Aug 2013
Wei Rochelle, Bhattacharya Arunabh, Hamilton Ryan T, Jernigan Amanda L, Chaudhuri Asish R
Abstract excerpt
Protein misfolding is considered to be a potential contributing factor for motor neuron and muscle loss in diseases like Amyotrophic lateral sclerosis (ALS). Several independent studies have demonstrated using over-expressed mutated Cu/Zn-superoxide dismutase (mSOD1) transgenic mouse models which mimic familial ALS (f-ALS), that both muscle and motor neurons undergo degeneration during disease progression....
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