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Transcriptional profiles for distinct aggregation states of mutant Huntingtin exon 1 protein unmask new Huntington's disease pathways

Moily, Nagaraj S; Ormsby, Angelique R; Stojilovic, Aleksandar; Ramdzan, Yasmin M; Diesch, Jeannine; Hannan, Ross D; Zajac, Michelle S; Hannan, Anthony J; Oshlack, Alicia; Hatters, Danny M

Description

Huntington's disease is caused by polyglutamine (polyQ)-expansion mutations in the CAG tandem repeat of the Huntingtin gene. The central feature of Huntington's disease pathology is the aggregation of mutant Huntingtin (Htt) protein into micrometer-sized inclusion bodies. Soluble mutant Htt states are most proteotoxic and trigger an enhanced risk of death whereas inclusions confer different changes to cellular health, and may even provide adaptive responses to stress. Yet the molecular...[Show more]

CollectionsANU Research Publications
Date published: 2017-09
Type: Journal article
URI: http://hdl.handle.net/1885/139047
Source: Molecular and cellular neurosciences
DOI: 10.1016/j.mcn.2017.07.004

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