Difference between revisions of "Ehinger 2016 Mov Disord"
m (moved Ciammola 2016 Mov Disord to Ehinger 2016 Mov Disord) |
|||
Line 1: | Line 1: | ||
{{Publication | {{Publication | ||
|title= | |title=Ehinger JK, Morota S, Hansson MJ, Gesine P, ElmĆ©r E (2016) Mitochondrial respiratory function in peripheral blood cells from Huntingtonās disease patients. Mov Disord doi:10.1002/mdc3.12308. | ||
|info=[http:// | |info=[http://onlinelibrary.wiley.com/doi/10.1002/mdc3.12308/abstract] | ||
|authors= | |authors=Ehinger JK, Morota S, Hansson MJ, Gesine P, Elmer E | ||
|year=2016 | |year=2016 | ||
|journal=Mov Disord | |journal=Mov Disord | ||
|abstract=Mitochondrial | |abstract=Patients with Huntingtonās 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 Huntingtonās 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. | ||
Ā | |||
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. | |||
Ā | |||
Normalized to citrate synthase activity, platelets from patients with Huntingtonās 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. | |||
Ā | |||
The data indicate decreased function of mitochondrial complex I in peripheral blood cells from patients with Huntingtonās 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=Huntingtonās disease, Mitochondria, Blood cells, Respirometry, Oxygen consumption | |||
|mipnetlab=JP Tokyo Uchino H, SE Lund Elmer E | |||
}} | }} | ||
{{Labeling | {{Labeling | ||
|area=Respiration, Exercise physiology;nutrition;life style, Patients | |area=Respiration, Exercise physiology;nutrition;life style, Patients | ||
|organism=Human | |organism=Human | ||
|tissues= | |tissues=Blood cells | ||
|model cell lines=Platelet | |model cell lines=Platelet | ||
|preparations=Intact cells, Permeabilized cells | |preparations=Intact cells, Permeabilized cells |
Revision as of 10:29, 21 March 2016
Ehinger JK, Morota S, Hansson MJ, Gesine P, ElmĆ©r E (2016) Mitochondrial respiratory function in peripheral blood cells from Huntingtonās disease patients. Mov Disord doi:10.1002/mdc3.12308. |
Ā» [1]
Ehinger JK, Morota S, Hansson MJ, Gesine P, Elmer E (2016) Mov Disord
Abstract: Patients with Huntingtonās 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 Huntingtonās 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 Huntingtonās 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 Huntingtonās 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. ā¢ Keywords: Huntingtonās disease, Mitochondria, Blood cells, Respirometry, Oxygen consumption
ā¢ 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
Labels, 2016-03