Huntington's disease mice and human brain tissue exhibit increased G3BP1 granules and TDP43 mislocalization.

ABSTRACT: Chronic cellular stress associated with neurodegenerative disease can result in the persistence of stress granule (SG) structures, membraneless organelles that form in response to cellular stress. In Huntington's disease (HD), chronic expression of mutant huntingtin generates various forms of cellular stress, including activation of the unfolded protein response and oxidative stress. However, it has yet to be determined whether SGs are a feature of HD neuropathology. We examined the miRNA composition of extracellular vesicles (EVs) present in the cerebrospinal fluid (CSF) of patients with HD and show that a subset of their target mRNAs were differentially expressed in the prefrontal cortex. Of these targets, SG components were enriched, including the SG-nucleating Ras GTPase-activating pro

SUBMITTER: Sanchez II 

PROVIDER: S-EPMC8203471 | biostudies-literature | 2021 Jun

REPOSITORIES: biostudies-literature