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Joag, H. and Ghatpande, V. and Desai, M. and Sarkar, M. and Raina, A. and Shinde, M. and Chitale, R. and Deo, A. and Bose, T. and Majumdar, A. (2019) A role of cellular translation regulation associated withtoxic Huntingtin protein. Cellular and Molecular Life Sciences.

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Huntington’s disease (HD) is a severe neurodegenerative disorder caused by poly Q repeat expansion in the Huntingtin (Htt) gene. While the Htt amyloid aggregates are known to affect many cellular processes, their role in translation has not been addressed. Here we report that pathogenic Htt expression causes a protein synthesis deficit in cells. We find a functional prion-like protein, the translation regulator Orb2, to be sequestered by Htt aggregates in cells. Co-expression of Orb2 can partially rescue the lethality associated with poly Q expanded Htt. These findings can be relevant for HD as human homologs of Orb2 are also sequestered by pathogenic Htt aggregates. Our work suggests that translation dysfunction is one of the contributors to the pathogenesis of HD and new therapies targeting protein synthesis pathways might help to alleviate disease symptoms.

Item Type: Article
Depositing User: Mr. Rameshwar Nema
Date Deposited: 25 Feb 2020 05:08
Last Modified: 25 Feb 2020 05:08
URI: http://nccs.sciencecentral.in/id/eprint/776

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