Novel oxysterols observed in tissues and fluids of AY9944-treated rats: a model for Smith-Lemli-Opitz syndrome
Treatment of Sprague-Dawley rats with AY9944, an inhibitor of 3β-hydroxysterol-Δ 7-
reductase (Dhcr7), leads to elevated levels of 7-dehydrocholesterol (7-DHC) and reduced
levels of cholesterol in all biological tissues, mimicking the key biochemical hallmark of
Smith-Lemli-Opitz syndrome (SLOS). Fourteen 7-DHC-derived oxysterols previously have
been identified as products of free radical oxidation in vitro; one of these oxysterols, 3β, 5α-
dihydroxycholest-7-en-6-one (DHCEO), was recently identified in Dhcr7-deficient cells and …
reductase (Dhcr7), leads to elevated levels of 7-dehydrocholesterol (7-DHC) and reduced
levels of cholesterol in all biological tissues, mimicking the key biochemical hallmark of
Smith-Lemli-Opitz syndrome (SLOS). Fourteen 7-DHC-derived oxysterols previously have
been identified as products of free radical oxidation in vitro; one of these oxysterols, 3β, 5α-
dihydroxycholest-7-en-6-one (DHCEO), was recently identified in Dhcr7-deficient cells and …