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IUBMB Comments Highly specific for 3β,7α-dihydroxy-C27 5
Synonyms
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3beta-hydroxy-DELTA5-C27-steroid oxidoreductase
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5-cholestene-3beta,7alpha-diol 3beta-dehydrogenase
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cholest-5-ene-3beta,7alpha-diol + NAD+ = 7alpha-hydroxycholest-4-en-3-one + NADH + H+
Highly specific for 3beta-hydroxy-C27-steroids with DELTA5-double bond
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MetaCyc
bile acid biosynthesis, neutral pathway
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cholest-5-ene-3beta,7alpha-diol:NAD+ 3-oxidoreductase
Highly specific for 3beta,7alpha-dihydroxy-C27-steroids with Delta5-double bond.
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cholest-5-ene-3beta,7alpha-diol + NAD+
7alpha-hydroxycholest-4-en-3-one + NADH
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Substrates: highly specific
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cholest-5-ene-3beta,7alpha-diol + NAD+
7alpha-hydroxycholest-4-en-3-one + NADH
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Substrates: highly specific
r
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NAD+
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can not be replaced by NADP+
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FMN
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30% inhibition at 0.1 mM
Quinacrine
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slight inhibition at 1 mM
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cholest-5-ene-3beta,7alpha-diol 3beta-dehydrogenase deficiency
Molecular genetics of 3beta-hydroxy-Delta5-C27-steroid oxidoreductase deficiency in 16 patients with loss of bile acid synthesis and liver disease.
Liver Diseases
Molecular genetics of 3beta-hydroxy-Delta5-C27-steroid oxidoreductase deficiency in 16 patients with loss of bile acid synthesis and liver disease.
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0.045
cholest-5-ene-3beta,7alpha-diol
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7 - 7.5
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in potassium phosphate buffer
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rabbit
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brenda
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Highest Expressing Human Cell Lines
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Cell Line Links
Gene Links
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monomer
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1 * 46000, SDS-PAGE
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additional information
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molecular analysis of 15 patients with neonatal cholestasis reveals 12 different mutations in HSD3B7 gene, 10 of them leading to complete inactivation of enzyme
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to homogeneity, chromatography techniques
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medicine
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molecular analysis of 15 patients with neonatal cholestasis reveals 12 different mutations in HSD3B7 gene, 10 of them leading to complete inactivation of enzyme
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Wikvall, K.
Purification and properties of a 3beta-hydroxy-DELTA5-C27-steroid oxidoreductase from rabbit liver microsomes
J. Biol. Chem.
256
3376-3380
1981
Oryctolagus cuniculus
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Cheng, J.B.; Jacquemin, E.; Gerhardt, M.; Nazer, H.; Cresteil, D.; Heubi, J.E.; Setchell, K.D.; Russell, D.W.
Molecular genetics of 3beta-hydroxy-DELTA5-C27-steroid oxidoreductase deficiency in 16 patients with loss of bile acid synthesis and liver disease
J. Clin. Endocrinol. Metab.
88
1833-1841
2003
Homo sapiens
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