Abstract
Huntington’s disease (HD) is a dominant debilitating neurodegenerative disorder caused by CAG trinucleotide repeat expansion in the huntingtin gene (HTT) on chromosome 4 of the human genome. The CAG repeat is highly polymorphic and varies from 6 to 35 in unaffected individuals and more than 35 repeats in HD patients. The encoded polyglutamine (polyQ) expansion in the exon 1 of the HTT gene that confers altered property to the protein leading to HD toxicity. However, in addition to the expansion of the polyQ stretch as a major cause of HD, recent reports suggest the involvement of post-translational modifications (PTMs) in metabolism, protein-protein interactions, and cellular toxicity. Various types of PTMs regulate protein stability, localization, function, and their interaction with other molecules and these have been reported in HD pathogenesis. Cleavage and clearance of mutant Htt (mHtt) and its interaction with other cellular processes are the key events leading to HD, and therefore, a better understanding of signaling pathways implicated in Htt protein modification is a mandate in the management of HD pathogenesis.
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