Oxysterols and Retinal Degeneration in a Rat Model of Smith-Lemli-Opitz Syndrome: Implications for an Improved Therapeutic Intervention

Smith-Lemli-Opitz syndrome (SLOS) is an autosomal recessive human disease caused by mutations in the gene encoding 7-dehydrocholesterol (7DHC) reductase (DHCR7), resulting in abnormal accumulation of 7DHC and reduced levels of cholesterol in bodily tissues and fluids. A rat model of the disease has...

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Bibliographic Details
Main Authors: Steven J. Fliesler, Libin Xu
Format: Article
Language:English
Published: MDPI AG 2018-10-01
Series:Molecules
Subjects:
Online Access:http://www.mdpi.com/1420-3049/23/10/2720