Ehinger 2016 Mov Disord

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Ehinger JK, Morota S, Hansson MJ, Gesine P, Elmér E (2016) Mitochondrial respiratory function in peripheral blood cells from Huntington’s disease patients. Mov Disord doi:10.1002/mdc3.12308.

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Ehinger JK, Morota S, Hansson MJ, Gesine P, Elmer E (2016) Mov Disord

Abstract: Patients with Huntington’s disease display symptoms from both the central nervous system and peripheral tissues. Mitochondrial dysfunction has been implicated as part of the pathogenesis of the disease and has been reported in brain tissue and extracerebral tissues, such as muscle and blood cells, but the results are inconsistent. Therefore, the authors performed a refined evaluation of mitochondrial function in 2 types of peripheral blood cells from 14 patients with Huntington’s disease and 21 control subjects. Several hypotheses were predefined, including impaired mitochondrial complex II function (primary), complex I function (secondary), and maximum oxidative phosphorylation capacity (secondary) in patient cells.

High-resolution respirometry was applied to viable platelets and mononuclear cells. Data were normalized to cell counts, citrate synthase activity, and mitochondrial DNA copy numbers.

Normalized to citrate synthase activity, platelets from patients with Huntington’s disease displayed respiratory dysfunction linked to complex I, complex II, and lower maximum oxidative phosphorylation capacity. No difference was seen in mononuclear cells or when platelet data were normalized to cell counts or mitochondrial DNA. The ratio of complex I respiration through maximum oxidative phosphorylation was significantly decreased in patients compared with controls. The corresponding ratio for complex II was unaffected.

The data indicate decreased function of mitochondrial complex I in peripheral blood cells from patients with Huntington’s disease, although this could not be uniformly confirmed. The results do not confirm a systemic complex II dysfunction and do not currently support the use of mitochondrial function in blood cells as a biomarker for the disease.

Keywords: Huntington’s disease, Mitochondria, Blood cells, Respirometry, Oxygen consumption, PBMC

O2k-Network Lab: JP Tokyo Uchino H, SE Lund Elmer E


Labels: MiParea: Respiration, Patients  Pathology: Neurodegenerative 

Organism: Human  Tissue;cell: Blood cells, Lymphocyte, Platelet  Preparation: Intact cells, Permeabilized cells 


Coupling state: LEAK, ROUTINE, OXPHOS, ET  Pathway: N, S, CIV, NS, Other combinations, ROX  HRR: Oxygraph-2k 

2016-03