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Article

Defective mitochondrial disulfide relay system, altered mitochondrial morphology and function in Huntington's disease.

Human molecular genetics - 1 Mar 2013

Napoli Eleonora, Wong Sarah, Hung Connie, Ross-Inta Catherine, Bomdica Prithvi, Giulivi Cecilia

Abstract excerpt

A number of studies have been conducted that link mitochondrial dysfunction (MD) to Huntington's disease (HD); however, contradicting results had resulted in a lack of a clear mechanism that links expression of mutant Huntingtin protein and MD. Mouse homozygous (HM) and heterozygous (HT) mutant striatal cells with two or one allele encoding for a mutant huntingtin protein with 111 polyGln repeats showed a...

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PMID: 23197653
DOI: 10.1093/hmg/dds503
PMCID: PMC8482967

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