| http://purl.uniprot.org/citations/26945068 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/26945068 | http://www.w3.org/2000/01/rdf-schema#comment | "Proteostasis maintenance of γ-aminobutyric acid type A (GABAA) receptors dictates their function in controlling neuronal inhibition in mammalian central nervous systems. However, as a multisubunit, multispan, integral membrane protein, even wild type subunits of GABAA receptors fold and assemble inefficiently in the endoplasmic reticulum (ER). Unassembled and misfolded subunits undergo ER-associated degradation (ERAD), but this degradation process remains poorly understood for GABAA receptors. Here, using the α1 subunits of GABAA receptors as a model substrate, we demonstrated that Grp94, a metazoan-specific Hsp90 in the ER lumen, uses its middle domain to interact with the α1 subunits and positively regulates their ERAD. OS-9, an ER-resident lectin, acts downstream of Grp94 to further recognize misfolded α1 subunits in a glycan-dependent manner. This delivers misfolded α1 subunits to the Hrd1-mediated ubiquitination and the valosin-containing protein-mediated extraction pathway. Repressing the initial ERAD recognition step by inhibiting Grp94 enhances the functional surface expression of misfolding-prone α1(A322D) subunits, which causes autosomal dominant juvenile myoclonic epilepsy. This study clarifies a Grp94-mediated ERAD pathway for GABAA receptors, which provides a novel way to finely tune their function in physiological and pathophysiological conditions."xsd:string |
| http://purl.uniprot.org/citations/26945068 | http://purl.org/dc/terms/identifier | "doi:10.1074/jbc.m115.705004"xsd:string |
| http://purl.uniprot.org/citations/26945068 | http://purl.uniprot.org/core/author | "Wang Y.J."xsd:string |
| http://purl.uniprot.org/citations/26945068 | http://purl.uniprot.org/core/author | "Fu Y.L."xsd:string |
| http://purl.uniprot.org/citations/26945068 | http://purl.uniprot.org/core/author | "Han D.Y."xsd:string |
| http://purl.uniprot.org/citations/26945068 | http://purl.uniprot.org/core/author | "Mu T.W."xsd:string |
| http://purl.uniprot.org/citations/26945068 | http://purl.uniprot.org/core/author | "Duerfeldt A.S."xsd:string |
| http://purl.uniprot.org/citations/26945068 | http://purl.uniprot.org/core/author | "Blagg B.S."xsd:string |
| http://purl.uniprot.org/citations/26945068 | http://purl.uniprot.org/core/author | "Di X.J."xsd:string |
| http://purl.uniprot.org/citations/26945068 | http://purl.uniprot.org/core/date | "2016"xsd:gYear |
| http://purl.uniprot.org/citations/26945068 | http://purl.uniprot.org/core/name | "J Biol Chem"xsd:string |
| http://purl.uniprot.org/citations/26945068 | http://purl.uniprot.org/core/pages | "9526-9539"xsd:string |
| http://purl.uniprot.org/citations/26945068 | http://purl.uniprot.org/core/title | "Grp94 Protein Delivers gamma-Aminobutyric Acid Type A (GABAA) Receptors to Hrd1 Protein-mediated Endoplasmic Reticulum-associated Degradation."xsd:string |
| http://purl.uniprot.org/citations/26945068 | http://purl.uniprot.org/core/volume | "291"xsd:string |
| http://purl.uniprot.org/citations/26945068 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/26945068 |
| http://purl.uniprot.org/citations/26945068 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/26945068 |
| http://purl.uniprot.org/uniprot/#_B4DHT9-mappedCitation-26945068 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26945068 |
| http://purl.uniprot.org/uniprot/#_B4DU71-mappedCitation-26945068 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26945068 |
| http://purl.uniprot.org/uniprot/#_B3KX82-mappedCitation-26945068 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26945068 |
| http://purl.uniprot.org/uniprot/#_B4DQX3-mappedCitation-26945068 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26945068 |
| http://purl.uniprot.org/uniprot/#_Q86TM6-mappedCitation-26945068 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26945068 |
| http://purl.uniprot.org/uniprot/#_Q16251-mappedCitation-26945068 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26945068 |
| http://purl.uniprot.org/uniprot/#_P14625-mappedCitation-26945068 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26945068 |
| http://purl.uniprot.org/uniprot/#_Q5CAQ5-mappedCitation-26945068 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26945068 |