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

• Published in: Folia biologica Vol. 64; no. 1; pp. 31 - 34
• Main Authors: Dušek, P, Rodinová, M, Lišková, I, Klempíř, J, Zeman, J, Roth, J, Hansíková, H
• Format: Journal Article
• Language: English
• Published: Czech Republic Charles University in Prague, First Faculty of Medicine 2018
• Subjects: Adenine; Age; Alzheimer's disease; Body mass index; Cachexia - metabolism; Calcium; Cardiomyopathy; Case-Control Studies; Citrate synthase; Cytosine; Electron transport; Electron Transport Complex I - metabolism; Electron Transport Complex IV - metabolism; Female; Gene expression; Guanine; Humans; Huntingtin; Huntington Disease - metabolism; Huntington's disease; Huntingtons disease; Male; Middle Aged; Mitochondria; Mouth Mucosa - metabolism; NADH; NADH dehydrogenase; Oxidative phosphorylation; Oxidative stress; PGC-1 protein; Phosphorylation; Proteinase inhibitors; Proteins; Studies; Trinucleotide repeats; St Louis Missouri; United States > US
• ISSN: 0015-5500; 2533-7602
• DOI: 10.14712/fb2018064010031

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.