Ontology highlight
ABSTRACT:
SUBMITTER: Seo H
PROVIDER: S-EPMC1800909 | biostudies-literature | 2007 Feb
REPOSITORIES: biostudies-literature
Seo Hyemyung H Sonntag Kai-Christian KC Kim Woori W Cattaneo Elena E Isacson Ole O
PloS one 20070228 2
In patients with Huntington's disease (HD), the proteolytic activity of the ubiquitin proteasome system (UPS) is reduced in the brain and other tissues. The pathological hallmark of HD is the intraneuronal nuclear protein aggregates of mutant huntingtin. We determined how to enhance UPS function and influence catalytic protein degradation and cell survival in HD. Proteasome activators involved in either the ubiquitinated or the non-ubiquitinated proteolysis were overexpressed in HD patients' ski ...[more]