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

@article{Napoli2013DefectiveMD,
  title={Defective mitochondrial disulfide relay system, altered mitochondrial morphology and function in Huntington's disease.},
  author={Eleonora Napoli and Sarah Wong and Connie K Hung and Catherine M Ross-Inta and Prithvi R. Bomdica and Cecilia Giulivi},
  journal={Human molecular genetics},
  year={2013},
  volume={22 5},
  pages={989-1004}
}
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 significant impairment of the mitochondrial disulfide relay system (MDRS). This system… CONTINUE READING

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