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Mitochondrial Dysfunction in Huntington's disease: Pathogenesis and Therapeutic Opportunities.

Authors:
Aditi Sharma Tapan Behl Lalit Sharma Lotfi Aelya Simona Bungau

Curr Drug Targets 2021 Feb 23. Epub 2021 Feb 23.

Department of Pharmacy, Faculty of Pharmacy, University of Oradea. Romania.

Huntington's disease (HD) is prototypical neurodegenerative disease, preferentially disrupts the neurons of striatum and cor-tex. Progressive motor dysfunctions, psychiatric disturbances, behavioural impairments and cognitive decline are the clinical symptoms of HD progression. The disease occurs due to, expanded CAG repeats in exon 1 of huntingtin protein (mHtt) causing its aggregation. Multiple cellular and molecular pathways are involved in the HD pathology. Mitochondria as vital organelles has an important role in most of the neurodegenerative diseases like HD. Over the years, the role of mitochondria in neurons are highly diverged, it not only contribute as cell power source, but as a dynamic organelles it fragments and then fuse to attain a maximal bioenergetics performance, regulate intracellular calcium homeostasis, reactive oxygen species (ROS) generation, antioxidant activity and involved in apoptotic pathways. Indeed, these events are seen to be affected in HD, resulting in neuronal dysfunction in pre-symptomatic stages. mHtt causes critical transcriptional abnormality by altering the expression of a master co-regulator, peroxisome proliferator-activated receptor gamma coactivator-1α (PGC-1α), leading to increased susceptibility to oxidative stress and neuronal degeneration. Moreover, mHtt influences multiple cellular signal-ling events which ends with mitochondrial biogenesis. Here, we resume recent findings that pose mitochondria as an im-portant regulatory organelle in HD and how mHtt affects mitochondrial function, trafficking and homeostasis and makes neurons prone to degeneration. In addition, we also uncover the mitochondrial based potential targets and therapeutic ap-proaches with imminent or currently ongoing clinical trials.

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Source
http://dx.doi.org/10.2174/1389450122666210224105945DOI Listing
February 2021

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