Fol. Biol. 2018, 64, 31-34

https://doi.org/10.14712/fb2018064010031

Buccal Respiratory Chain Complexes I and IV Quantities in Huntington’s Disease Patients

P. Dušek1, M. Rodinová2, I. Lišková1, J. Klempíř1,3, J. Zeman2, J. Roth1, Hana Hansíková2

1Department of Neurology and Centre of Clinical Neuroscience, First Faculty of Medicine, Charles University and General University Hospital in Prague, Czech Republic
2Laboratory for the Study of Mitochondrial Disorders, Department of Paediatrics and Adolescent Medicine, First Faculty of Medicine, Charles University and General University Hospital in Prague, Czech Republic
3Institute of Anatomy, First Faculty of Medicine, Charles University and General University Hospital in Prague, Czech Republic

Received February 2018
Accepted March 2018

Alterations in mitochondrial parameters are an important hallmark of Huntington’s disease (HD). The ubiquitous expression of mutant huntingtin raises the prospect that mitochondrial disturbances can also be detected and monitored through buccal epithelial cells. In a group of 34 patients with Huntington’s disease and a group of 22 age-related healthy volunteers, respiratory complex I and IV protein quantities in buccal epithelial cells were measured using the dipstick immunocapture assay. The protein quantity of respiratory complex I correlates with age (r = 0.427, P = 0.026, FWE-P = 0.156) in the patient group, but not in the group of healthy subjects. Our non-invasive approach allows us to obtain valuable information for the studies of mitochondrial biochemical parameters in patients with neurodegenerative diseases and could also be useful in epidemiological studies.

Funding

This study was supported by COST LD15099, PROGRES Q26/ 1LF and PROGRES Q27/1LF.

References

31 live references