Ontology highlight
ABSTRACT:
SUBMITTER: Zhang S
PROVIDER: S-EPMC6994494 | biostudies-literature | 2020 Jan
REPOSITORIES: biostudies-literature
Zhang Sunyuan S Hinde Elizabeth E Parkyn Schneider Molly M Jans David A DA Bogoyevitch Marie A MA
Scientific reports 20200131 1
A mutant form of the ataxin-1 protein with an expanded polyglutamine (polyQ) tract is the underlying cause of the inherited neurodegenerative disease spinocerebellar ataxia 1 (SCA1). In probing the biophysical features of the nuclear bodies (NBs) formed by polyQ-ataxin-1, we defined ataxin-1 NBs as spherical liquid protein/RNA droplets capable of rapid fusion. We observed dynamic exchange of the ataxin-1 protein into these NBs; notably, cell exposure to a pro-oxidant stress could trigger a trans ...[more]