Extensive respiratory chain defects in inhibitory interneurones in patients with mitochondrial disease

Neuropathology and Applied Neurobiology
Nichola Z LaxDoug M Turnbull

Abstract

Mitochondrial disorders are among the most frequently inherited cause of neurological disease and arise due to mutations in mitochondrial or nuclear DNA. Currently, we do not understand the specific involvement of certain brain regions or selective neuronal vulnerability in mitochondrial disease. Recent studies suggest γ-aminobutyric acid (GABA)-ergic interneurones are particularly susceptible to respiratory chain dysfunction. In this neuropathological study, we assess the impact of mitochondrial DNA defects on inhibitory interneurones in patients with mitochondrial disease. Histochemical, immunohistochemical and immunofluorescent assays were performed on post-mortem brain tissue from 10 patients and 10 age-matched control individuals. We applied a quantitative immunofluorescent method to interrogate complex I and IV protein expression in mitochondria within GABAergic interneurone populations in the frontal, temporal and occipital cortices. We also evaluated the density of inhibitory interneurones in serial sections to determine if cell loss was occurring. We observed significant, global reductions in complex I expression within GABAergic interneurones in frontal, temporal and occipital cortices in the majority of patients. Whi...Continue Reading

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Citations

Nov 26, 2015·Annals of Neurology·Anne GrünewaldDoug M Turnbull
Aug 18, 2017·Neurology. Genetics·Yi Shiau NgMarios Hadjivassiliou
Jun 12, 2016·Neuropathology and Applied Neurobiology·N Z LaxD M Turnbull
Nov 3, 2016·The Journal of Pathology·Charlotte L AlstonRobert W Taylor
Sep 5, 2015·Neuropathology and Applied Neurobiology·Alexia ChrysostomouNichola Z Lax
Feb 25, 2020·Wellcome Open Research·Yi Shiau NgDoug M Turnbull
Jul 11, 2020·Acta Neuropathologica Communications·Christopher HattonDaniel Erskine
Oct 30, 2020·The American Journal of Psychiatry·Jill R GlausierDavid A Lewis
Feb 28, 2021·Cellular and Molecular Neurobiology·Xu ZhouLili Cui
Jun 1, 2021·Journal of Neural Transmission·Daniel Erskine, Johannes Attems
Jul 3, 2021·Children·Dorota Wesół-KucharskaAleksandra Jezela-Stanek

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