RDF/XMLNTriplesTurtleShow queryShare
SubjectPredicateObject
http://purl.uniprot.org/citations/19158078http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/19158078http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/19158078http://www.w3.org/2000/01/rdf-schema#comment"Rapsyn is a postsynaptic protein required for clustering of nicotinic acetylcholine receptors (nAChRs) at the neuromuscular junction. Here we report the mechanism for posttranslational control of rapsyn protein stability. We confirmed that C18H9.7-encoded RPY-1 is a rapsyn homolog in Caenorhabditis elegans by showing that human rapsyn rescued rpy-1 mutant phenotypes in nematodes, as determined by levamisole assays and micropost array behavioral assays. We found that RPY-1 was degraded in the absence of functional UNC-29, a non-alpha subunit of the receptor, in an allele-specific manner, but not in the absence of other receptor subunits. The cytoplasmic loop of UNC-29 was found to be critical for RPY-1 stability. Through RNA interference screening, we found that UBC-1, UBC-12, NEDD-8, and RBX-1 were required for degradation of RPY-1. We identified cullin (CUL)-3 as a component of E3 ligase and KEL-8 as the substrate adaptor of RPY-1. Mammalian rapsyn was ubiquitinated by the CUL3/KLHL8-containing E3 ligase in vitro, and the knockdown of KLHL-8, a mammalian KEL-8 homolog, inhibited rapsyn ubiquitination in vivo, implying evolutionary conservation of the rapsyn stability control machinery. kel-8 suppression and rpy-1 overexpression in C. elegans produced a phenotype similar to that of a loss-of-function mutation of rpy-1, suggesting that control of rapsyn abundance is important for proper function of the receptor. Our results suggest a link between the control of rapsyn abundance and congenital myasthenic syndromes."xsd:string
http://purl.uniprot.org/citations/19158078http://purl.org/dc/terms/identifier"doi:10.1074/jbc.m808230200"xsd:string
http://purl.uniprot.org/citations/19158078http://purl.org/dc/terms/identifier"doi:10.1074/jbc.m808230200"xsd:string
http://purl.uniprot.org/citations/19158078http://purl.uniprot.org/core/author"Lee J.H."xsd:string
http://purl.uniprot.org/citations/19158078http://purl.uniprot.org/core/author"Lee J.H."xsd:string
http://purl.uniprot.org/citations/19158078http://purl.uniprot.org/core/author"Lee H."xsd:string
http://purl.uniprot.org/citations/19158078http://purl.uniprot.org/core/author"Lee H."xsd:string
http://purl.uniprot.org/citations/19158078http://purl.uniprot.org/core/author"Lee J."xsd:string
http://purl.uniprot.org/citations/19158078http://purl.uniprot.org/core/author"Lee J."xsd:string
http://purl.uniprot.org/citations/19158078http://purl.uniprot.org/core/author"Park S."xsd:string
http://purl.uniprot.org/citations/19158078http://purl.uniprot.org/core/author"Park S."xsd:string
http://purl.uniprot.org/citations/19158078http://purl.uniprot.org/core/author"Hwang H."xsd:string
http://purl.uniprot.org/citations/19158078http://purl.uniprot.org/core/author"Hwang H."xsd:string
http://purl.uniprot.org/citations/19158078http://purl.uniprot.org/core/author"Min K."xsd:string
http://purl.uniprot.org/citations/19158078http://purl.uniprot.org/core/author"Min K."xsd:string
http://purl.uniprot.org/citations/19158078http://purl.uniprot.org/core/author"Nam S."xsd:string
http://purl.uniprot.org/citations/19158078http://purl.uniprot.org/core/author"Nam S."xsd:string
http://purl.uniprot.org/citations/19158078http://purl.uniprot.org/core/author"Yoon J."xsd:string
http://purl.uniprot.org/citations/19158078http://purl.uniprot.org/core/author"Yoon J."xsd:string
http://purl.uniprot.org/citations/19158078http://purl.uniprot.org/core/author"Lee H.O."xsd:string
http://purl.uniprot.org/citations/19158078http://purl.uniprot.org/core/author"Lee H.O."xsd:string
http://purl.uniprot.org/citations/19158078http://purl.uniprot.org/core/date"2009"xsd:gYear
http://purl.uniprot.org/citations/19158078http://purl.uniprot.org/core/date"2009"xsd:gYear