Document Detail


Dysmorphic photoreceptors in a P23H mutant rhodopsin model of retinitis pigmentosa are metabolically active and capable of regenerating to reverse retinal degeneration.
MedLine Citation:
PMID:  22323724     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
This study evaluated the capacity of Xenopus laevis retina to regenerate photoreceptor cells after cyclic light-mediated acute rod photoreceptor degeneration in a transgenic P23H mutant rhodopsin model of retinits pigmentosa. After discontinuation of cyclic light exposure, we monitored histologic progression of retinal regeneration over a 3 week recovery period. To assess their metabolomic states, contralateral eyes were processed for computational molecular phenotyping. We found that retinal degeneration in the P23H rhodopsin mutation could be partially reversed, with regeneration of rod photoreceptors recovering normal morphology (including full-length rod outer segments) by the end of the 3 week recovery period. In contrast, retinal degeneration mediated by directly induced apoptosis did not recover in the 3 week recovery period. Dystrophic rod photoreceptors with truncated rod outer segments were identified as the likely source of rod photoreceptor regeneration in the P23H retinas. These dystrophic photoreceptors remain metabolically active despite having lost most of their outer segments.
Authors:
Damian C Lee; Felix R Vazquez-Chona; W Drew Ferrell; Beatrice M Tam; Bryan W Jones; Robert E Marc; Orson L Moritz
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The Journal of neuroscience : the official journal of the Society for Neuroscience     Volume:  32     ISSN:  1529-2401     ISO Abbreviation:  J. Neurosci.     Publication Date:  2012 Feb 
Date Detail:
Created Date:  2012-02-10     Completed Date:  2012-03-27     Revised Date:  2012-05-02    
Medline Journal Info:
Nlm Unique ID:  8102140     Medline TA:  J Neurosci     Country:  United States    
Other Details:
Languages:  eng     Pagination:  2121-8     Citation Subset:  IM    
Affiliation:
Department of Ophthalmology and Visual Sciences and Centre for Macular Research, University of British Columbia, Vancouver, British Columbia, Canada, V5Z 3N9.
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MeSH Terms
Descriptor/Qualifier:
Amino Acid Substitution* / genetics
Animals
Animals, Genetically Modified
Disease Models, Animal
Histidine / genetics
Mutation* / genetics
Nerve Regeneration / genetics
Proline / genetics
Retinal Degeneration / genetics*,  metabolism
Retinal Rod Photoreceptor Cells / metabolism*,  physiology
Retinitis Pigmentosa / genetics*,  metabolism
Rhodopsin / genetics,  metabolism*,  physiology
Xenopus laevis
Grant Support
ID/Acronym/Agency:
5T32 HD07491/HD/NICHD NIH HHS; EY014800/EY/NEI NIH HHS; EY015128/EY/NEI NIH HHS; EY02576/EY/NEI NIH HHS; P30 EY014800/EY/NEI NIH HHS; P30 EY014800-08/EY/NEI NIH HHS; R01 EY002576-36/EY/NEI NIH HHS; R01 EY015128-07/EY/NEI NIH HHS; //Canadian Institutes of Health Research
Chemical
Reg. No./Substance:
147-85-3/Proline; 71-00-1/Histidine; 9009-81-8/Rhodopsin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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