Article
Mitochondrial dysfunction in Huntington's disease: the bioenergetics of isolated and in situ mitochondria from transgenic mice.
Journal of neurochemistry - 1 Apr 2007
Oliveira Jorge M A, Jekabsons Mika B, Chen Sylvia, Lin Amy, Rego A Cristina, Gonçalves Jorge, Ellerby Lisa M, Nicholls David G
Abstract excerpt
Mitochondrial dysfunction is believed to participate in Huntington's disease (HD) pathogenesis. Here we compare the bioenergetic behavior of forebrain mitochondria isolated from different transgenic HD mice (R6/2, YAC128 and Hdh150 knock-in) and wild-type littermates with the first determination of in situ respiratory parameters in intact HD striatal neurons. We assess the Ca2+-loading capacity of isolated...
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