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Difference between revisions of "Ehinger 2016 Mov Disord"

From Bioblast
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|tissues=Skeletal muscle, Blood cells
|tissues=Skeletal muscle, Blood cells
|model cell lines=Platelet
|model cell lines=Platelet
|preparations=Intact cells, Permeabilized cells
|diseases=Neurodegenerative
|diseases=Neurodegenerative
|couplingstates=LEAK, ROUTINE, OXPHOS, ETS
|substratestates=CI, CII, FAO, CIV, CI&II, Other combinations, ROX
|instruments=Oxygraph-2k
|instruments=Oxygraph-2k
|additional=Labels, 2016-03
|additional=Labels, 2016-03
}}
}}

Revision as of 16:25, 18 March 2016

Publications in the MiPMap
Ciammola A, Sassone J, Sciacco M, Mencacci NE, Ripolone M, Bizzi C, Colciago C, Moggio M, Parati G, Silani V, Malfatto G (2016) Low anaerobic threshold and increased skeletal muscle lactate production in subjects with Huntington's disease. Mov Disord 26:130-7.

Β» PMID: 20931633 Open Access

Ciammola A, Sassone J, Sciacco M, Mencacci NE, Ripolone M, Bizzi C, Colciago C, Moggio M, Parati G, Silani V, Malfatto G (2016) Mov Disord

Abstract: Mitochondrial defects that affect cellular energy metabolism have long been implicated in the etiology of Huntington's disease (HD). Indeed, several studies have found defects in the mitochondrial functions of the central nervous system and peripheral tissues of HD patients. In this study, we investigated the in vivo oxidative metabolism of exercising muscle in HD patients. Ventilatory and cardiometabolic parameters and plasma lactate concentrations were monitored during incremental cardiopulmonary exercise in twenty-five HD subjects and twenty-five healthy subjects. The total exercise capacity was normal in HD subjects but notably the HD patients and presymptomatic mutation carriers had a lower anaerobic threshold than the control subjects. The low anaerobic threshold of HD patients was associated with an increase in the concentration of plasma lactate. We also analyzed in vitro muscular cell cultures and found that HD cells produce more lactate than the cells of healthy subjects. Finally, we analyzed skeletal muscle samples by electron microscopy and we observed striking mitochondrial structural abnormalities in two out of seven HD subjects. Our findings confirm mitochondrial abnormalities in HD patients' skeletal muscle and suggest that the mitochondrial dysfunction is reflected functionally in a low anaerobic threshold and an increased lactate synthesis during intense physical exercise.


Labels: MiParea: Respiration, Exercise physiology;nutrition;life style, Patients  Pathology: Neurodegenerative 

Organism: Human  Tissue;cell: Skeletal muscle, 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