Matches in SemOpenAlex for { <https://semopenalex.org/work/W3214972596> ?p ?o ?g. }
Showing items 1 to 86 of
86
with 100 items per page.
- W3214972596 abstract "Dehydrodolichyl diphosphate synthase (DHDDS) catalyzes the commitment 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 wk compared to age-matched controls; however, rapid photoreceptor degeneration ensued, resulting in almost complete loss of rods and cones by PN 6 wk. Retina dolichol levels were markedly decreased by PN 4 wk in Dhddsflx/flx iCre+ mice, relative to controls; despite this, N-glycosylation of retinal proteins, including rhodopsin (the dominant rod-specific glycoprotein), persisted in Dhddsflx/flx iCre+ mice. These findings challenge the conventional mechanistic view of RP59 as a congenital disease of glycosylation (CDG)." @default.
- W3214972596 created "2021-12-06" @default.
- W3214972596 creator A5000476110 @default.
- W3214972596 creator A5009178683 @default.
- W3214972596 creator A5011764176 @default.
- W3214972596 creator A5015659453 @default.
- W3214972596 creator A5022591492 @default.
- W3214972596 creator A5031960520 @default.
- W3214972596 creator A5039367843 @default.
- W3214972596 creator A5049381929 @default.
- W3214972596 creator A5050319998 @default.
- W3214972596 creator A5063284895 @default.
- W3214972596 creator A5068006897 @default.
- W3214972596 creator A5074635677 @default.
- W3214972596 creator A5090897169 @default.
- W3214972596 date "2020-01-01" @default.
- W3214972596 modified "2023-09-27" @default.
- W3214972596 title "Retinal Degeneration Caused by Rod-Specific <i>Dhdds</i> Ablation Without Concomitant Inhibition of Protein <i>N</i>-Glycosylation" @default.
- W3214972596 doi "https://doi.org/10.2139/ssrn.3543841" @default.
- W3214972596 hasPublicationYear "2020" @default.
- W3214972596 type Work @default.
- W3214972596 sameAs 3214972596 @default.
- W3214972596 citedByCount "0" @default.
- W3214972596 crossrefType "journal-article" @default.
- W3214972596 hasAuthorship W3214972596A5000476110 @default.
- W3214972596 hasAuthorship W3214972596A5009178683 @default.
- W3214972596 hasAuthorship W3214972596A5011764176 @default.
- W3214972596 hasAuthorship W3214972596A5015659453 @default.
- W3214972596 hasAuthorship W3214972596A5022591492 @default.
- W3214972596 hasAuthorship W3214972596A5031960520 @default.
- W3214972596 hasAuthorship W3214972596A5039367843 @default.
- W3214972596 hasAuthorship W3214972596A5049381929 @default.
- W3214972596 hasAuthorship W3214972596A5050319998 @default.
- W3214972596 hasAuthorship W3214972596A5063284895 @default.
- W3214972596 hasAuthorship W3214972596A5068006897 @default.
- W3214972596 hasAuthorship W3214972596A5074635677 @default.
- W3214972596 hasAuthorship W3214972596A5090897169 @default.
- W3214972596 hasConcept C126322002 @default.
- W3214972596 hasConcept C134018914 @default.
- W3214972596 hasConcept C153911025 @default.
- W3214972596 hasConcept C169760540 @default.
- W3214972596 hasConcept C181199279 @default.
- W3214972596 hasConcept C202033177 @default.
- W3214972596 hasConcept C2777093970 @default.
- W3214972596 hasConcept C2777313579 @default.
- W3214972596 hasConcept C2780827179 @default.
- W3214972596 hasConcept C2781040256 @default.
- W3214972596 hasConcept C2781114197 @default.
- W3214972596 hasConcept C2781446604 @default.
- W3214972596 hasConcept C553450214 @default.
- W3214972596 hasConcept C55493867 @default.
- W3214972596 hasConcept C71924100 @default.
- W3214972596 hasConcept C86803240 @default.
- W3214972596 hasConceptScore W3214972596C126322002 @default.
- W3214972596 hasConceptScore W3214972596C134018914 @default.
- W3214972596 hasConceptScore W3214972596C153911025 @default.
- W3214972596 hasConceptScore W3214972596C169760540 @default.
- W3214972596 hasConceptScore W3214972596C181199279 @default.
- W3214972596 hasConceptScore W3214972596C202033177 @default.
- W3214972596 hasConceptScore W3214972596C2777093970 @default.
- W3214972596 hasConceptScore W3214972596C2777313579 @default.
- W3214972596 hasConceptScore W3214972596C2780827179 @default.
- W3214972596 hasConceptScore W3214972596C2781040256 @default.
- W3214972596 hasConceptScore W3214972596C2781114197 @default.
- W3214972596 hasConceptScore W3214972596C2781446604 @default.
- W3214972596 hasConceptScore W3214972596C553450214 @default.
- W3214972596 hasConceptScore W3214972596C55493867 @default.
- W3214972596 hasConceptScore W3214972596C71924100 @default.
- W3214972596 hasConceptScore W3214972596C86803240 @default.
- W3214972596 hasLocation W32149725961 @default.
- W3214972596 hasOpenAccess W3214972596 @default.
- W3214972596 hasPrimaryLocation W32149725961 @default.
- W3214972596 hasRelatedWork W1051562 @default.
- W3214972596 hasRelatedWork W10937782 @default.
- W3214972596 hasRelatedWork W318235 @default.
- W3214972596 hasRelatedWork W369672 @default.
- W3214972596 hasRelatedWork W5356161 @default.
- W3214972596 hasRelatedWork W5944761 @default.
- W3214972596 hasRelatedWork W8746921 @default.
- W3214972596 hasRelatedWork W927837 @default.
- W3214972596 hasRelatedWork W9290317 @default.
- W3214972596 hasRelatedWork W9930405 @default.
- W3214972596 isParatext "false" @default.
- W3214972596 isRetracted "false" @default.
- W3214972596 magId "3214972596" @default.
- W3214972596 workType "article" @default.