Article
The effect of SOD1 mutation on cellular bioenergetic profile and viability in response to oxidative stress and influence of mutation-type.
PloS one - 1 Jan 2013
Richardson Katie, Allen Scott P, Mortiboys Heather, Grierson Andrew J, Wharton Stephen B, Ince Paul G, Shaw Pamela J, Heath Paul R
Abstract excerpt
Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disorder characterized by the progressive degeneration of motor neurons. Substantial evidence implicates oxidative stress and mitochondrial dysfunction as early events in disease progression. Our aim was to ascertain whether mutation of the SOD1 protein increases metabolic functional susceptibility to oxidative stress. Here we used a motor...
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