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A37 Buccal respiratory chain complexes I and IV quantities in huntington’s disease patients
  1. Pavel Dusek1,
  2. Marie Rodinova2,
  3. Irena Liskova1,
  4. Jiri Klempir1,3,
  5. Jiri Zeman2,
  6. Jan Roth1,
  7. Hana Hansikova2
  1. 1Department of Neurology and Center of Clinical Neuroscience, First Faculty of Medicine, Charles University and General University Hospital in Prague, Czech Republic
  2. 2Laboratory for the Study of Mitochondrial Disorders, First Faculty of Medicine, Charles University and General University Hospital in Prague, Czech Republic
  3. 3Institute of Anatomy, First Faculty of Medicine, Charles University and General University Hospital in Prague, Czech Republic

Abstract

Alterations in mitochondrial parameteres are an important hallmark of Huntington’s disease (HD). Given the ubiquitous expression of mutant huntingtin raises the prospect that mitochondrial disturbancies can be detected and monitored also in 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 dipstick immunocapture assay. The protein quantity of respiratory complex I is correlated 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 to obtain valuable materials for studies on mitochondrial biochemical parameters in patients with neurodegenerative diseases and could be be useful in epidemiological studies.

  • mitochondria
  • buccal epithelium
  • dipstick immunoassay

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