Document Detail

Lipid hydroperoxide formation in the retina: correlation with retinal degeneration and light damage in a rat model of Smith-Lemli-Opitz syndrome.
MedLine Citation:
PMID:  16199034     Owner:  NLM     Status:  MEDLINE    
The Smith-Lemli-Opitz syndrome (SLOS) is an autosomal recessive disease presenting with multiple congenital anomalies, caused by a defect in cholesterol biosynthesis that results in abnormally elevated levels of 7-dehydrocholesterol (7DHC). Progressive retinal degeneration has been demonstrated in a rat model of SLOS, which is markedly exacerbated by intense light, far more so than occurs in normal albino rats under the same conditions. Herein, we demonstrate that, by six postnatal weeks, retinas in the SLOS rat model contain levels of lipid hydroperoxides (LPOs) comparable to those found in light-damaged albino rats (twice the normal steady-state levels), and that intense light exposure results in a three-fold elevation of LPOs with concomitant severe retinal degeneration. These results suggest a correlation between retinal degeneration and LPO levels. We propose that the presence of 7DHC in the SLOS rat retina potentiates LPO formation, and promotes the observed hypersensitivity to light-induced retinal degeneration.
Michael J Richards; Barbara A Nagel; Steven J Fliesler
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2005-09-30
Journal Detail:
Title:  Experimental eye research     Volume:  82     ISSN:  0014-4835     ISO Abbreviation:  Exp. Eye Res.     Publication Date:  2006 Mar 
Date Detail:
Created Date:  2006-01-30     Completed Date:  2006-04-11     Revised Date:  2014-09-15    
Medline Journal Info:
Nlm Unique ID:  0370707     Medline TA:  Exp Eye Res     Country:  England    
Other Details:
Languages:  eng     Pagination:  538-41     Citation Subset:  IM    
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MeSH Terms
Light / adverse effects
Lipid Peroxides / biosynthesis*
Models, Animal
Rats, Sprague-Dawley
Retina / metabolism*,  pathology
Retinal Degeneration / metabolism*,  pathology
Smith-Lemli-Opitz Syndrome / metabolism*,  pathology
Grant Support
EY07361/EY/NEI NIH HHS; R01 EY007361/EY/NEI NIH HHS; R01 EY007361-14A2/EY/NEI NIH HHS
Reg. No./Substance:
0/Lipid Peroxides

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

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