The chicken or the egg: mitochondrial dysfunction as a cause or consequence of toxicity in Huntington’s disease

Polyzos, Aris A.; McMurray, Cynthia T.

doi:10.1016/j.mad.2016.09.003

Title: The chicken or the egg: mitochondrial dysfunction as a cause or consequence of toxicity in Huntington’s disease

Journal Article · Mon Sep 12 00:00:00 EDT 2016 · Mechanisms of Ageing and Development

DOI:https://doi.org/10.1016/j.mad.2016.09.003· OSTI ID:1393099

^[1]; McMurray, Cynthia T. ^[1]

Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States). Molecular Biophysics and Integrated Bioimaging Division

Mitochondrial dysfunction and ensuing oxidative damage is typically thought to be a primary cause of Huntington's disease, Alzheimer's disease, and Parkinson disease. There is little doubt that mitochondria (MT) become defective as neurons die, yet whether MT defects are the primary cause or a detrimental consequence of toxicity remains unanswered. Oxygen consumption rate (OCR) and glycolysis provide sensitive and informative measures of the functional status MT and the cells metabolic regulation, yet these measures differ depending on the sample source; species, tissue type, age at measurement, and whether MT are measured in purified form or in a cell. The effects of these various parameters are difficult to quantify and not fully understood, but clearly have an impact on interpreting the bioenergetics of MT or their failure in disease states. A major goal of the review is to discuss issues and coalesce detailed information into a reference table to help in assessing mitochondrial dysfunction as a cause or consequence of Huntington's disease.

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Research Organization:: Lawrence Berkeley National Laboratory (LBNL), Berkeley, CA (United States)

Sponsoring Organization:: National Institutes of Health (NIH)

Grant/Contract Number:: AC02-05CH11231; NS060115; CA092584

OSTI ID:: 1393099

Journal Information:: Mechanisms of Ageing and Development, Vol. 161, Issue Part A; ISSN 0047-6374

Publisher:: ElsevierCopyright Statement

Country of Publication:: United States

Language:: English

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Cited by: 19 works

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Guidelines on experimental methods to assess mitochondrial dysfunction in cellular models of neurodegenerative diseases Connolly, Niamh M. C.; Theurey, Pierre; Adam-Vizi, Vera Cell Death & Differentiation, Vol. 25, Issue 3 https://doi.org/10.1038/s41418-017-0020-4	journal	December 2017
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Comparison of Sirtuin 3 Levels in ALS and Huntington’s Disease—Differential Effects in Human Tissue Samples vs. Transgenic Mouse Models Buck, Eva; Bayer, Hanna; Lindenberg, Katrin S. Frontiers in Molecular Neuroscience, Vol. 10 https://doi.org/10.3389/fnmol.2017.00156	journal	May 2017
Guidelines on experimental methods to assess mitochondrial dysfunction in cellular models of neurodegenerative diseases. Connolly, Niamh MC; Theurey, Pierre; Adam-Vizi, Vera Apollo - University of Cambridge Repository https://doi.org/10.17863/cam.24241	text	January 2018

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