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Property "Has-20abstract" (as page type) with input value "Metabolic-20dysfunction-20and-20mitochondrial-20involvement-20are-20recognised-20as-20part-20of-20the-20pathology-20in-20Huntington's-20Disease-20(HD).-20Post-2Dmortem-20examinations-20of-20the-20striatum-20from-20end-2Dstage-20HD-20patients-20have-20shown-20a-20decrease-20in-20the-20-27-27in-20vitro-27-27-20activity-20of-20Complexes-20II,-20III-20and-20IV-20of-20the-20electron-20transfer-2Dpathway-20(ET-2Dpathway).-20In-20different-20models-20of-20HD,-20evidence-20of-20enzyme-20defects-20have-20been-20reported-20in-20Complex-20II-20and-20Complex-20IV-20using-20enzyme-20assays.-20However,-20such-20assays-20are-20highly-20variable-20and-20results-20have-20been-20inconsistent.-20We-20investigated-20the-20integrated-20ET-2Dpathway-20function-20ex-20vivo-20using-20a-20sensitive-20high-2Dresolution-20respirometric-20(HRR)-20method.-20The-20O2-20flux-20in-20a-20whole-2Dcell-20sample-20combined-20with-20the-20addition-20of-20mitochondrial-20substrates,-20uncouplers-20and-20inhibitors-20enabled-20us-20to-20accurately-20quantitate-20the-20function-20of-20inspanidual-20mitochondrial-20complexes-20in-20intact-20mitochondria,-20while-20retaining-20mitochondrial-20regulation-20and-20compensatory-20mechanisms.-20We-20used-20HRR-20to-20examine-20the-20mitochondrial-20function-20in-20striata-20from-2012-2Dweek-20old-20R6-2F2-20mice-20expressing-20exon-201-20of-20human-20HTT-20with-20130-20CAG-20repeats.-20A-20significant-20reduction-20in-20Complex-20II-20and-20Complex-20IV-20flux-20control-20ratios-20was-20found-20in-20the-20R6-2F2-20mouse-20striatum-20at-2012-20weeks-20of-20age-20compared-20to-20controls,-20confirming-20previous-20findings-20obtained-20with-20spectrophotometric-20enzyme-20assays." contains invalid characters or is incomplete and therefore can cause unexpected results during a query or annotation process.

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