Matches in SemOpenAlex for { <https://semopenalex.org/work/W2113669792> ?p ?o ?g. }
Showing items 1 to 93 of
93
with 100 items per page.
- W2113669792 endingPage "197" @default.
- W2113669792 startingPage "191" @default.
- W2113669792 abstract "The mechanism of autosomal dominant retinitis pigmentosa (ADRP) caused by the P23H mutation in rhodopsin is tightly associated with misfolded rhodopsin (RHO) which causes endoplasmic reticulum overload (ER stress), activates the unfolded protein response (UPR), and triggers apoptosis. In efforts to create a therapy for ADRP caused by the P23H mutation, we have explored different approaches leading to survival of photoreceptor (PR) cells. The direct approach involves the modulation of the level of wild-type RHO, while the indirect approach involves reprogramming the UPR and increasing the expression of heat shock proteins (HSPs). Taking the direct approach, we found that overexpression of wild-type RHO rescues scotopic ERG responses partially. However, greater therapeutic effects were obtained by manipulation of the UPR in P23H RHO rat PRs treated with the endoplasmic reticulum protein BiP/Grp78. In vitro study revealed that the prosurvival effect of Bip gene was not associated with its function as a molecular chaperone, but rather with its regulation of the UPR. Another indirect approach was the overexpression of the Hsf-1 gene, a transcriptional regulator of the heat shock response. AAV-delivery of Hsf-1 resulted in an increase of scotopic ERG amplitudes by over 35%. Taken together, these data suggest viable therapeutic treatments for ADRP." @default.
- W2113669792 created "2016-06-24" @default.
- W2113669792 creator A5003383828 @default.
- W2113669792 creator A5021299820 @default.
- W2113669792 creator A5040674203 @default.
- W2113669792 creator A5076578673 @default.
- W2113669792 creator A5081401643 @default.
- W2113669792 creator A5091772109 @default.
- W2113669792 date "2011-11-11" @default.
- W2113669792 modified "2023-09-26" @default.
- W2113669792 title "Functional Rescue of P23H Rhodopsin Photoreceptors by Gene Delivery" @default.
- W2113669792 cites W2005186288 @default.
- W2113669792 cites W2008220319 @default.
- W2113669792 cites W2039602145 @default.
- W2113669792 cites W2076759214 @default.
- W2113669792 cites W2118468592 @default.
- W2113669792 cites W2137101268 @default.
- W2113669792 doi "https://doi.org/10.1007/978-1-4614-0631-0_26" @default.
- W2113669792 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/3365920" @default.
- W2113669792 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/22183333" @default.
- W2113669792 hasPublicationYear "2011" @default.
- W2113669792 type Work @default.
- W2113669792 sameAs 2113669792 @default.
- W2113669792 citedByCount "23" @default.
- W2113669792 countsByYear W21136697922013 @default.
- W2113669792 countsByYear W21136697922014 @default.
- W2113669792 countsByYear W21136697922015 @default.
- W2113669792 countsByYear W21136697922016 @default.
- W2113669792 countsByYear W21136697922017 @default.
- W2113669792 countsByYear W21136697922018 @default.
- W2113669792 countsByYear W21136697922019 @default.
- W2113669792 countsByYear W21136697922020 @default.
- W2113669792 countsByYear W21136697922021 @default.
- W2113669792 countsByYear W21136697922022 @default.
- W2113669792 countsByYear W21136697922023 @default.
- W2113669792 crossrefType "book-chapter" @default.
- W2113669792 hasAuthorship W2113669792A5003383828 @default.
- W2113669792 hasAuthorship W2113669792A5021299820 @default.
- W2113669792 hasAuthorship W2113669792A5040674203 @default.
- W2113669792 hasAuthorship W2113669792A5076578673 @default.
- W2113669792 hasAuthorship W2113669792A5081401643 @default.
- W2113669792 hasAuthorship W2113669792A5091772109 @default.
- W2113669792 hasBestOaLocation W21136697922 @default.
- W2113669792 hasConcept C104317684 @default.
- W2113669792 hasConcept C139447449 @default.
- W2113669792 hasConcept C142724271 @default.
- W2113669792 hasConcept C158617107 @default.
- W2113669792 hasConcept C202033177 @default.
- W2113669792 hasConcept C205260736 @default.
- W2113669792 hasConcept C2775962898 @default.
- W2113669792 hasConcept C2780827179 @default.
- W2113669792 hasConcept C2781114197 @default.
- W2113669792 hasConcept C54355233 @default.
- W2113669792 hasConcept C55493867 @default.
- W2113669792 hasConcept C71924100 @default.
- W2113669792 hasConcept C86803240 @default.
- W2113669792 hasConcept C95444343 @default.
- W2113669792 hasConceptScore W2113669792C104317684 @default.
- W2113669792 hasConceptScore W2113669792C139447449 @default.
- W2113669792 hasConceptScore W2113669792C142724271 @default.
- W2113669792 hasConceptScore W2113669792C158617107 @default.
- W2113669792 hasConceptScore W2113669792C202033177 @default.
- W2113669792 hasConceptScore W2113669792C205260736 @default.
- W2113669792 hasConceptScore W2113669792C2775962898 @default.
- W2113669792 hasConceptScore W2113669792C2780827179 @default.
- W2113669792 hasConceptScore W2113669792C2781114197 @default.
- W2113669792 hasConceptScore W2113669792C54355233 @default.
- W2113669792 hasConceptScore W2113669792C55493867 @default.
- W2113669792 hasConceptScore W2113669792C71924100 @default.
- W2113669792 hasConceptScore W2113669792C86803240 @default.
- W2113669792 hasConceptScore W2113669792C95444343 @default.
- W2113669792 hasLocation W21136697921 @default.
- W2113669792 hasLocation W21136697922 @default.
- W2113669792 hasLocation W21136697923 @default.
- W2113669792 hasLocation W21136697924 @default.
- W2113669792 hasOpenAccess W2113669792 @default.
- W2113669792 hasPrimaryLocation W21136697921 @default.
- W2113669792 hasRelatedWork W1994289493 @default.
- W2113669792 hasRelatedWork W2072667779 @default.
- W2113669792 hasRelatedWork W2102337733 @default.
- W2113669792 hasRelatedWork W2809677173 @default.
- W2113669792 hasRelatedWork W2907310168 @default.
- W2113669792 hasRelatedWork W2910831402 @default.
- W2113669792 hasRelatedWork W2937188981 @default.
- W2113669792 hasRelatedWork W3092166485 @default.
- W2113669792 hasRelatedWork W3142122528 @default.
- W2113669792 hasRelatedWork W4311514635 @default.
- W2113669792 isParatext "false" @default.
- W2113669792 isRetracted "false" @default.
- W2113669792 magId "2113669792" @default.
- W2113669792 workType "book-chapter" @default.