Mitochondrial DNA levels in Huntington disease leukocytes and dermal fibroblasts

Metabolic Brain Disease
Paulina JędrakSylwia Barańska

Abstract

Huntington disease (HD) is an inherited neurodegenerative disorder caused by mutations in the huntingtin gene. Involvement of mitochondrial dysfunctions in, and especially influence of the level of mitochondrial DNA (mtDNA) on, development of this disease is unclear. Here, samples of blood from 84 HD patients and 79 controls, and dermal fibroblasts from 10 HD patients and 9 controls were analysed for mtDNA levels. Although the type of mitochondrial haplogroup had no influence on the mtDNA level, and there was no correlation between mtDNA level in leukocytes in HD patients and various parameters of HD severity, some considerable differences between HD patients and controls were identified. The average mtDNA/nDNA relative copy number was significantly higher in leukocytes, but lower in fibroblasts, of symptomatic HD patients relative to the control group. Moreover, HD women displayed higher mtDNA levels in leukocytes than HD men. Because this is the largest population analysed to date, these results might contribute to explanation of discrepancies between previously published studies concerning levels of mtDNA in cells of HD patients. We suggest that the size of the investigated population and type of cells from which DNA is isol...Continue Reading

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Dec 16, 2017·European Journal of Clinical Investigation·Anna PiccaFrancesco Russo
Dec 28, 2018·Nucleosides, Nucleotides & Nucleic Acids·Marta ToczekRyszard T Smolenski
Aug 5, 2020·Neurodegenerative Disease Management·Andreas Neueder, Michael Orth
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Mar 10, 2019·Metabolic Brain Disease·Karolina PierzynowskaGrzegorz Węgrzyn

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Methods Mentioned

BETA
biopsies
PCR

Software Mentioned

SAS
MITOMAP
PhyloTree

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