Article
Altered p53 and NOX1 activity cause bioenergetic defects in a SCA7 polyglutamine disease model.
Biochimica et biophysica acta - 1 Jan 2000
Ajayi Abiodun, Yu Xin, Wahlo-Svedin Carolina, Tsirigotaki Galateia, Karlström Victor, Ström Anna-Lena
Abstract excerpt
Spinocerebellar ataxia type 7 (SCA7) is one of the nine neurodegenerative disorders caused by expanded polyglutamine (polyQ) domains. Common pathogenic mechanisms, including bioenergetics defects, have been suggested for these so called polyQ diseases. However, the exact molecular mechanism(s) behind the metabolic dysfunction is still unclear. In this study we identified a previously unreported mechanism,...
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