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Retinal Degeneration in a Rodent Model of Smith-Lemli-Opitz Syndrome: Electrophysiologic, Biochemical, and Morphologic Features

OBJECTIVE: To assess the electrophysiologic, histologic, and biochemical features of an animal model of Smith-Lemli-Opitz syndrome (SLOS). METHODS: Sprague-Dawley rats were treated with AY9944, a selective inhibitor of 3β-hydroxysterol-Δ7-reductase (the affected enzyme in SLOS). Dark- and light-adap...

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Detalhes bibliográficos
Main Authors: Fliesler, Steven J., Peachey, Neal S., Richards, Michael J., Nagel, Barbara A., Vaughan, Dana K.
Formato: Artigo
Idioma:Inglês
Publicado em: 2004
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2865831/
https://ncbi.nlm.nih.gov/pubmed/15302661
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1001/archopht.122.8.1190
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