Article
Oxidative stress promotes mutant huntingtin aggregation and mutant huntingtin-dependent cell death by mimicking proteasomal malfunction.
Biochemical and biophysical research communications - 31 Mar 2006
Goswami Anand, Dikshit Priyanka, Mishra Amit, Mulherkar Shalaka, Nukina Nobuyuki, Jana Nihar Ranjan
Abstract excerpt
Huntington's disease (HD) is a familial neurodegenerative disorder caused by an abnormal expansion of CAG repeats in the coding region of huntingtin gene. A major hallmark of HD is the proteolytic production of N-terminal fragments of huntingtin containing polyglutamine repeats that form ubiquitinated aggregates in the nucleus and cytoplasm of the affected neurons. However, the mechanism by which the mutant...
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