Article
Quercitrin and quercetin 3-β-d-glucoside as chemical chaperones for the A4V SOD1 ALS-causing mutant.
Protein engineering, design & selection : PEDS - 1 Jun 2017
Ip Philbert, Sharda Priya Roy, Cunningham Anna, Chakrabartty Sumon, Pande Vijay, Chakrabartty Avijit
Abstract excerpt
In many cases of familial amyotrophic lateral sclerosis (ALS), mutant forms of the Cu,Zn superoxide dismutase protein (SOD1) misfold and aggregate in motor neurons. Monomers of the normally homodimeric SOD1 have been found in patient tissue, presymptomatic mouse models of ALS, and in vitro misfolding assays which suggests that monomerization might be an early step in the pathological SOD1 misfolding pathway. In...
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