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A gain-of-function of an amyotrophic lateral sclerosis-associated Cu,Zn-superoxide dismutase mutant: An enhancement of free radical formation due to a decrease in Km for hydrogen peroxide.

Cu,Zn-superoxide dismutase (SOD) is known to be a locus of mutation in familial amyotrophic lateral sclerosis (FALS). Transgenic mice that express a mutant Cu,Zn-SOD, Gly-93--> Ala (G93A), have been shown to develop amyotrophic lateral sclerosis (ALS) symptoms. We cloned the FALS mutant, G93A, and w...

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Udgivet i:Proc Natl Acad Sci U S A
Principais autores: Yim, M B, Kang, J H, Yim, H S, Kwak, H S, Chock, P B, Stadtman, E R
Format: Artigo
Sprog:Inglês
Udgivet: National Academy of Sciences 1996
Fag:
Research Article
Online adgang:https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC39125/
https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/8650157/
https://ncbi.nlm.nih.govhttps://doi.org/10.1073/pnas.93.12.5709
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