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Honda, A; Salen, G; Matsuzaki, Y et al. (2001) Differences in hepatic levels of intermediates in bile acid biosynthesis between Cyp27(-/-) mice and CTX. J Lipid Res 42:291-300
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Honda, A; Salen, G; Matsuzaki, Y et al. (2001) Side chain hydroxylations in bile acid biosynthesis catalyzed by CYP3A are markedly up-regulated in Cyp27-/- mice but not in cerebrotendinous xanthomatosis. J Biol Chem 276:34579-85
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Honda, M; Tint, G S; Honda, A et al. (2000) Regulation of cholesterol biosynthetic pathway in patients with the Smith-Lemli-Opitz syndrome. J Inherit Metab Dis 23:464-74
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Honda, A; Salen, G; Shefer, S et al. (2000) Regulation of 25- and 27-hydroxylation side chain cleavage pathways for cholic acid biosynthesis in humans, rabbits, and mice. Assay of enzyme activities by high-resolution gas chromatography;-mass spectrometry. J Lipid Res 41:442-51
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Honda, A; Salen, G; Honda, M et al. (2000) 3-Hydroxy-3-methylglutaryl-coenzyme A reductase activity is inhibited by cholesterol and up-regulated by sitosterol in sitosterolemic fibroblasts. J Lab Clin Med 135:174-9
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Honda, A; Salen, G; Shefer, S et al. (1999) Bile acid synthesis in the Smith-Lemli-Opitz syndrome: effects of dehydrocholesterols on cholesterol 7alpha-hydroxylase and 27-hydroxylase activities in rat liver. J Lipid Res 40:1520-8
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Nguyen, L B; Shefer, S; Salen, G et al. (1998) Competitive inhibition of hepatic sterol 27-hydroxylase by sitosterol: decreased activity in sitosterolemia. Proc Assoc Am Physicians 110:32-9
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Honda, A; Salen, G; Nguyen, L B et al. (1998) Regulation of early cholesterol biosynthesis in rat liver: effects of sterols, bile acids, lovastatin, and BM 15.766 on 3-hydroxy-3-methylglutaryl coenzyme A synthase and acetoacetyl coenzyme A thiolase activities. Hepatology 27:154-9
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Honda, A; Salen, G; Nguyen, L B et al. (1998) Down-regulation of cholesterol biosynthesis in sitosterolemia: diminished activities of acetoacetyl-CoA thiolase, 3-hydroxy-3-methylglutaryl-CoA synthase, reductase, squalene synthase, and 7-dehydrocholesterol delta7-reductase in liver and mononuclear leu J Lipid Res 39:44-50
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Honda, M; Tint, G S; Shefer, S et al. (1998) Accurate detection of Smith-Lemli-Opitz syndrome carriers by measurement of the rate of reduction of the ergosterol C-7 double bond in cultured skin fibroblasts. J Inherit Metab Dis 21:761-8
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