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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 significant impairment of the mitochondrial disulfide relay system (MDRS). This system (consisting of two proteins, Gfer and Mia40) is involved in the mitochondrial import of Cys-rich proteins. The Gfer-to-Mia40 ratio was significantly altered in HM cells compared with controls, along with the expression of mitochondrial proteins considered substrates of the MDRS. In progenitors and differentiated neuron-like HM cells, impairment of MDRS were accompanied by deficient oxidative phosphorylation, Complex I, IV and V activities, decreased mtDNA copy number and transcripts, accumulation of mtDNA deletions and changes in mitochondrial morphology, consistent with other MDRS-deficient biological models, thus providing a framework for the energy deficits observed in this HD model. The majority (&amp;gt;90%) of the mitochondrial outcomes exhibited a gene\u2013dose dependency with the expression of mutant Htt. Finally, decreases in the mtDNA copy number, along with the accumulation of mtDNA deletions, provide a mechanism for the progressive neurodegeneration observed in HD patients.<\/jats:p>","DOI":"10.1093\/hmg\/dds503","type":"journal-article","created":{"date-parts":[[2012,11,30]],"date-time":"2012-11-30T04:28:15Z","timestamp":1354249695000},"page":"989-1004","source":"Crossref","is-referenced-by-count":69,"title":["Defective mitochondrial disulfide relay system, altered mitochondrial morphology and function in Huntington's disease"],"prefix":"10.1093","volume":"22","author":[{"given":"Eleonora","family":"Napoli","sequence":"first","affiliation":[{"name":"Department of Molecular Biosciences, University of California Davis, Davis, CA 95616, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Wong","sequence":"additional","affiliation":[{"name":"Department of Molecular Biosciences, University of California Davis, Davis, CA 95616, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Connie","family":"Hung","sequence":"additional","affiliation":[{"name":"Department of Molecular Biosciences, University of California Davis, Davis, CA 95616, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"Ross-Inta","sequence":"additional","affiliation":[{"name":"Department of Molecular Biosciences, University of California Davis, Davis, CA 95616, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Prithvi","family":"Bomdica","sequence":"additional","affiliation":[{"name":"Department of Molecular Biosciences, University of California Davis, Davis, CA 95616, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cecilia","family":"Giulivi","sequence":"additional","affiliation":[{"name":"Department of Molecular Biosciences, University of California Davis, Davis, CA 95616, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2012,11,29]]},"reference":[{"key":"2021092109053199800_DDS503C1","doi-asserted-by":"crossref","first-page":"1267","DOI":"10.1056\/NEJM198611133152006","article-title":"Huntington's disease. 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