Mitochondrial matters of the brain: the role in Huntington’s disease

@article{Turner2010MitochondrialMO,
  title={Mitochondrial matters of the brain: the role in Huntington’s disease},
  author={Christopher Turner and Anthony H V Schapira},
  journal={Journal of Bioenergetics and Biomembranes},
  year={2010},
  volume={42},
  pages={193-198}
}
  • C. Turner, A. Schapira
  • Published 18 May 2010
  • Biology, Medicine
  • Journal of Bioenergetics and Biomembranes
Even before the discovery of the mutant htt gene as the cause of Huntington’s Disease (HD), abnormal energy metabolism and mitochondrial dysfunction had been suggested as a possible pathogenic mechanism in HD. These initial investigations described defects in energy metabolism using Positron Emission Tomography (PET) and Nuclear Magnetic Resonance (NMR) Spectroscopy in symptomatic and pre-symptomatic HD patients. Concurrently, 3-nitroproprionic acid, a mitochondrial complex II inhibitor, was… 
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TLDR
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TLDR
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TLDR
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Recent progress in SIRT1 research is summarized, with a focus on the specificity of this protein as a potential therapeutic target for HD, as well as existing challenges for developing Sirtuin-1 modulators for clinical use.
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TLDR
It is proposed to consider VDAC as a crucial element in HD etiology and consequently as a reasonable target for therapeutic interventions in HD, based on developing novel therapeutic strategies eliminating mitochondria dysfunction.
Impaired mitochondrial oxidative phosphorylation in the peroxisomal disease X-linked adrenoleukodystrophy.
TLDR
The data indicate that in X-ALD patients' fibroblasts, excess of C26:0 generates mtDNA oxidation and specifically impairs oxidative phosphorylation (OXPHOS) triggering mitochondrial ROS production from electron transport chain complexes, leading to a multifaceted dysfunction of this organelle.
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TLDR
The mitochondrial defect identified in HD caudate parallels that induced by HD neurotoxin models and further supports the role of abnormal energy metabolism in HD.
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TLDR
Investigation of respiratory chain function in HD skeletal muscle provides additional evidence that mutant huntingtin influences mitochondrial complex II/III function in non‐neuronal tissue (skeletal muscle) and suggests that muscle may be a potential marker of disease progression in HD.
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TLDR
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TLDR
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TLDR
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TLDR
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TLDR
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TLDR
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