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Current Drug Metabolism

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ISSN (Print): 1389-2002
ISSN (Online): 1875-5453

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Review Article

A Review On Huntington Protein: Insight Into Protein Aggregation and Therapeutic Interventions

Author(s): Ekambaram Srinivasan, Vavish Ram and Ramalingam Rajasekaran*

Volume 23, Issue 4, 2022

Published on: 25 May, 2022

Page: [260 - 282] Pages: 23

DOI: 10.2174/1389200223666220321103942

Price: $65

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Abstract

Huntington's disease (HD) is a distressing, innate neurodegenerative disease that descends from CAG repeat expansion in the huntingtin gene causing behavioral changes, motor dysfunction, and dementia in children and adults. Mutation in huntingtin (HTT) protein has been suggested to cause neuron loss in the cortex and striatum through various mechanisms, including abnormal regulation of transcription, proteasomal dysfunction, posttranslational modification, and other events regulating toxicity. Pathogenesis of HD involves cleavage of the huntingtin protein followed by the neuronal accumulation of its aggregated form. Several research groups made possible efforts to reduce huntingtin gene expression, protein accumulation, and protein aggregation using inhibitors and molecular chaperones as developing drugs against HD. Herein, we review the mechanism proposed towards the formation of HTT protein aggregation and the impact of therapeutic strategies for the treatment of HD.

Keywords: HTT mutation, protein aggregation, molecular chaperons, huntington’s disease, neurodegenerative disease, dementia.

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