Mutant huntingtin fails to directly impair brain mitochondria.

ABSTRACT: Although the mechanisms by which mutant huntingtin (mHtt) results in Huntington's disease (HD) remain unclear, mHtt-induced mitochondrial defects were implicated in HD pathogenesis. The effect of mHtt could be mediated by transcriptional alterations, by direct interaction with mitochondria, or by both. In the present study, we tested a hypothesis that mHtt directly damages mitochondria. To test this hypothesis, we applied brain cytosolic fraction from YAC128 mice, containing mHtt, to brain non-synaptic and synaptic mitochondria from wild-type mice and assessed mitochondrial respiration with a Clark-type oxygen electrode, membrane potential and Ca²⁺ uptake capacity with tetraphenylphosphonium (TPP⁺ )- and Ca²⁺ -sensitive electrodes, respectively, and, reacti

SUBMITTER: Hamilton J 

PROVIDER: S-EPMC6917837 | biostudies-literature | 2019 Dec

REPOSITORIES: biostudies-literature