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A. Honda

University of Medicine and Dentistry of New Jersey

19

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606

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Side Chain Hydroxylations in Bile Acid Biosynthesis Catalyzed by CYP3A Are Markedly Up-regulated in Cyp27−/− Mice but Not in Cerebrotendinous Xanthomatosis

2001 Journal of Biological Chemistry
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Fetal demise with Smith-Lemli-Opitz syndrome confirmed by tissue sterol analysis and the absence of measurable 7-dehydrocholesterol Δ7-reductase activity in chorionic villi

2000 Prenatal Diagnosis
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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

1998 Journal of Inherited Metabolic Disease
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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 Δ7-reductase in liver and mononuclear leukocytes

1998 Journal of Lipid Research
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substrate specificity, competitive and non-competitive inhibition, and phosphorylation/dephosphorylation

1998
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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

1998 Hepatology
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Cholesterol and oxygenated cholesterol concentrations are markedly elevated in peripheral tissue but not in brain from mice with the Niemann–Pick type C phenotype

1998 Journal of Inherited Metabolic Disease
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Regulation of rat hepatic 3β-hydroxysterol Δ7-reductase: substrate specificity, competitive and non-competitive inhibition, and phosphorylation/dephosphorylation

1998 Journal of Lipid Research
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Sterol concentrations in cultured Smith-Lemli-Opitz syndrome skin fibroblasts : Diagnosis of a biochemically atypical case of the syndrome

1997 American Journal of Medical Genetics
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Synthesis of [3α-3H]7-dehydrocholesterol via stable tritiated 4-phenyl-1,2,4-triazoline-3,5-dione derivative

1997 Steroids
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