Ehinger 2016 Mov Disord
Ehinger JK, Morota S, Hansson MJ, Gesine P, Elmรฉr E (2016) Mitochondrial respiratory function in peripheral blood cells from Huntingtonโs disease patients. Mov Disord doi:10.1002/mdc3.12308. |
ยป [1]
Ehinger JK, Morota S, Hansson MJ, Gesine P, Elmer E (2016) Mov Disord
Abstract: Patients with Huntingtonโs 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 Huntingtonโs 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 Huntingtonโs 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 Huntingtonโs 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. โข Keywords: Huntingtonโs disease, Mitochondria, Blood cells, Respirometry, Oxygen consumption, PBMC
โข O2k-Network Lab: JP Tokyo Uchino H, SE Lund Elmer E
Labels: MiParea: Respiration, Exercise physiology;nutrition;life style, Patients
Pathology: Neurodegenerative
Organism: Human Tissue;cell: Blood cells Preparation: Intact cells, Permeabilized cells
Coupling state: LEAK, ROUTINE, OXPHOS, ETS"ETS" is not in the list (LEAK, ROUTINE, OXPHOS, ET) of allowed values for the "Coupling states" property.
HRR: Oxygraph-2k
2016-03