http://purl.uniprot.org/citations/32526701 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
http://purl.uniprot.org/citations/32526701 | http://www.w3.org/2000/01/rdf-schema#comment | "Dehydrodolichyl diphosphate synthase (DHDDS) catalyzes the committed step in dolichol synthesis. Recessive mutations in DHDDS cause retinitis pigmentosa (RP59), resulting in blindness. We hypothesized that rod photoreceptor-specific ablation of Dhdds would cause retinal degeneration due to diminished dolichol-dependent protein N-glycosylation. Dhddsflx/flx mice were crossed with rod-specific Cre recombinase-expressing (Rho-iCre75) mice to generate rod-specific Dhdds knockout mice (Dhddsflx/flx iCre+). In vivo morphological and electrophysiological evaluation of Dhddsflx/flx iCre+ retinas revealed mild retinal dysfunction at postnatal (PN) 4 weeks, compared with age-matched controls; however, rapid photoreceptor degeneration ensued, resulting in almost complete loss of rods and cones by PN 6 weeks. Retina dolichol levels were markedly decreased by PN 4 weeks in Dhddsflx/flx iCre+ mice, relative to controls; despite this, N-glycosylation of retinal proteins, including opsin (the dominant rod-specific glycoprotein), persisted in Dhddsflx/flx iCre+ mice. These findings challenge the conventional mechanistic view of RP59 as a congenital disorder of glycosylation."xsd:string |
http://purl.uniprot.org/citations/32526701 | http://purl.org/dc/terms/identifier | "doi:10.1016/j.isci.2020.101198"xsd:string |
http://purl.uniprot.org/citations/32526701 | http://purl.uniprot.org/core/author | "Wu F."xsd:string |
http://purl.uniprot.org/citations/32526701 | http://purl.uniprot.org/core/author | "Mu X."xsd:string |
http://purl.uniprot.org/citations/32526701 | http://purl.uniprot.org/core/author | "Swiezewska E."xsd:string |
http://purl.uniprot.org/citations/32526701 | http://purl.uniprot.org/core/author | "Surmacz L."xsd:string |
http://purl.uniprot.org/citations/32526701 | http://purl.uniprot.org/core/author | "Pittler S.J."xsd:string |
http://purl.uniprot.org/citations/32526701 | http://purl.uniprot.org/core/author | "Fliesler S.J."xsd:string |
http://purl.uniprot.org/citations/32526701 | http://purl.uniprot.org/core/author | "Butler M.C."xsd:string |
http://purl.uniprot.org/citations/32526701 | http://purl.uniprot.org/core/author | "Danikiewicz W."xsd:string |
http://purl.uniprot.org/citations/32526701 | http://purl.uniprot.org/core/author | "Spolnik G."xsd:string |
http://purl.uniprot.org/citations/32526701 | http://purl.uniprot.org/core/author | "Stacks D.A."xsd:string |
http://purl.uniprot.org/citations/32526701 | http://purl.uniprot.org/core/author | "Onysk A."xsd:string |
http://purl.uniprot.org/citations/32526701 | http://purl.uniprot.org/core/author | "Ramachandra Rao S."xsd:string |
http://purl.uniprot.org/citations/32526701 | http://purl.uniprot.org/core/author | "Skelton L.A."xsd:string |
http://purl.uniprot.org/citations/32526701 | http://purl.uniprot.org/core/date | "2020"xsd:gYear |
http://purl.uniprot.org/citations/32526701 | http://purl.uniprot.org/core/name | "iScience"xsd:string |
http://purl.uniprot.org/citations/32526701 | http://purl.uniprot.org/core/pages | "101198"xsd:string |
http://purl.uniprot.org/citations/32526701 | http://purl.uniprot.org/core/title | "Retinal Degeneration Caused by Rod-Specific Dhdds Ablation Occurs without Concomitant Inhibition of Protein N-Glycosylation."xsd:string |
http://purl.uniprot.org/citations/32526701 | http://purl.uniprot.org/core/volume | "23"xsd:string |
http://purl.uniprot.org/citations/32526701 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/32526701 |
http://purl.uniprot.org/citations/32526701 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/32526701 |
http://purl.uniprot.org/uniprot/#_A3KGK9-mappedCitation-32526701 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/32526701 |
http://purl.uniprot.org/uniprot/#_A3KGL0-mappedCitation-32526701 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/32526701 |