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Aidt 2013 PLoS Curr
Coupling states LEAK  + , OXPHOS  + , ET  +
Diseases Neurodegenerative  +
Enzyme TCA cycle and matrix dehydrogenases  +
Has abstract Metabolic dysfunction and mitochondrial in
Metabolic dysfunction and mitochondrial involvement are recognised as part of the pathology in Huntington's Disease (HD). Post-mortem examinations of the striatum from end-stage HD patients have shown a decrease in the ''in vitro'' activity of Complexes II, III and IV of the electron transfer-pathway (ET-pathway). In different models of HD, evidence of enzyme defects have been reported in Complex II and Complex IV using enzyme assays. However, such assays are highly variable and results have been inconsistent. We investigated the integrated ET-pathway function ex vivo using a sensitive high-resolution respirometric (HRR) method. The O2 flux in a whole-cell sample combined with the addition of mitochondrial substrates, uncouplers and inhibitors enabled us to accurately quantitate the function of individual mitochondrial complexes in intact mitochondria, while retaining mitochondrial regulation and compensatory mechanisms. We used HRR to examine the mitochondrial function in striata from 12-week old R6/2 mice expressing exon 1 of human HTT with 130 CAG repeats. A significant reduction in Complex II and Complex IV flux control ratios was found in the R6/2 mouse striatum at 12 weeks of age compared to controls, confirming previous findings obtained with spectrophotometric enzyme assays.
ned with spectrophotometric enzyme assays.  +
Has info [ PMID:23568011 Open Access]  +
Has title Aidt FH, Nielsen SM, Kanters J, Pesta D, N
Aidt FH, Nielsen SM, Kanters J, Pesta D, Nielsen TT, Nørremølle A, Hasholt L, Christiansen M, Hagen CM (2013) Dysfunctional mitochondrial respiration in the striatum of the Huntington's disease transgenic R6/2 mouse model. PLoS Curr 5. pii:ecurrents.hd.d8917b4862929772c5a2f2a34ef1c201.
rents.hd.d8917b4862929772c5a2f2a34ef1c201.  +
Instrument and method Oxygraph-2k  +
Mammal and model Mouse  +
MiP area Respiration  + , Genetic knockout;overexpression  +
Pathways N  + , S  + , CIV  + , NS  + , ROX  +
Preparation Homogenate  +
Respiration and regulation Uncoupler  +
Tissue and cell Nervous system  +
Was published by MiPNetLab DK Copenhagen Christiansen M +
Was published in journal PLoS Curr +
Was published in year 2013  +
Was written by Aidt FH + , Nielsen SM + , Kanters J + , Pesta D + , Nielsen TT + , Noerremoelle A + , Hasholt L + , Christiansen M + , Hagen CM +
Categories Publications
Modification date
"Modification date" is a predefined property that corresponds to the date of the last modification of a subject and is provided by Semantic MediaWiki.
19:21:22, 17 March 2019  +
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