Article

The molecular pathogenesis of superoxide dismutase 1-linked ALS is promoted by low oxygen tension

2018-05-11

Abstract excerpt

Mutations that destabilize superoxide dismutase 1 (SOD1) are a cause of amyotrophic lateral sclerosis (ALS). SOD1, which is located in the reducing cytosol, contains an oxidized disulfide bond required for stability. We show that the bond is an Achilles heel of the protein because it is sensitive to the oxygen tension. Culture of ALS patient-derived fibroblasts, astrocytes and induced pluripotent stem cell-derived...

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Literature Corpus work: 3619d5cb-c1ad-5612-8d62-e624d9121667
DOI: 10.1101/320283

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