Ontology highlight
ABSTRACT:
SUBMITTER: Dong Z
PROVIDER: S-EPMC218776 | biostudies-literature | 2003 Oct
REPOSITORIES: biostudies-literature
Dong Zhizhong Z Ferger Boris B Paterna Jean-Charles JC Vogel Denise D Furler Sven S Osinde Maribel M Feldon Joram J Büeler Hansruedi H
Proceedings of the National Academy of Sciences of the United States of America 20031006 21
Mutations in the parkin gene are linked to autosomal-recessive juvenile parkinsonism (AR-JP). Parkin functions as a ubiquitin protein ligase in the degradation of several proteins, including the neuron-specific septin CDCrel-1. AR-JP-associated parkin mutations inhibit ubiquitination and degradation of CDCrel-1 and other parkin target proteins. Here we show that recombinant adeno-associated virus-mediated CDCrel-1 gene transfer to the substantia nigra of rats results in a rapid onset (6-10 days) ...[more]