Retinal degeneration in a rodent model of Smith-Lemli-Opitz syndrome: electrophysiologic, biochemical, and morphologic features
SJ Fliesler, NS Peachey, MJ Richards… - Archives of …, 2004 - jamanetwork.com
Objective To assess the electrophysiologic, histologic, and biochemical featuresof an animal
model of Smith-Lemli-Opitz syndrome (SLOS). Methods Sprague-Dawley rats were treated
with AY9944, a selective inhibitorof 3β-hydroxysterol-Δ 7-reductase (the affected enzymein
SLOS). Dark-and light-adapted electroretinograms were obtained from treatedand control
animals. From each animal, 1 retina was analyzed by microscopy, and the contralateral
retina plus serum samples were analyzed for sterol composition. The main outcome …
model of Smith-Lemli-Opitz syndrome (SLOS). Methods Sprague-Dawley rats were treated
with AY9944, a selective inhibitorof 3β-hydroxysterol-Δ 7-reductase (the affected enzymein
SLOS). Dark-and light-adapted electroretinograms were obtained from treatedand control
animals. From each animal, 1 retina was analyzed by microscopy, and the contralateral
retina plus serum samples were analyzed for sterol composition. The main outcome …