A huntingtin-mediated fast stress response halting endosomal trafficking is defective in Huntington's disease.

ABSTRACT: Cellular stress is a normal part of the aging process and is especially relevant in neurodegenerative disease. Canonical stress responses, such as the heat shock response, activate following exposure to stress and restore proteostasis through the action of isomerases and chaperones within the cytosol. Through live-cell imaging, we demonstrate involvement of the Huntington's disease (HD) protein, huntingtin, in a rapid cell stress response that lies temporally upstream of canonical stress responses. This response is characterized by the formation of distinct cytosolic puncta and reversible localization of huntingtin to early endosomes. The formation of these puncta, which we have termed huntingtin stress bodies (HSBs), is associated with arrest of early-to-recycling and early-to-late endoso

SUBMITTER: Nath S 

PROVIDER: S-EPMC4275073 | biostudies-literature | 2015 Jan

REPOSITORIES: biostudies-literature