Cholest-5-ene-3beta,7alpha,27-triol is oxidized and isomerized to 4-cholesten-7alpha,27-diol-3-one

Stable Identifier
R-HSA-193816
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
Cholest-5-ene-3beta,7alpha,27-triol + NAD+ => 4-cholesten-7alpha,27-diol-3-one +NADH + H+
ReviewStatus
5/5
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Cholest-5-ene-3beta,7alpha,27-triol and NAD+ react to form 4-cholesten-7alpha,27-diol-3-one and NADH + H+, in a reaction catalyzed by HSD3B7 (3 beta-hydroxysteroid dehydrogenase type 7) in the endoplasmic reticulum membrane. Its function in vivo has been confirmed in studies of patients with defects in bile acid synthesis (Schwarz et al. 2000).
Literature References
PubMed ID Title Journal Year
11067870 The bile acid synthetic gene 3beta-hydroxy-Delta(5)-C(27)-steroid oxidoreductase is mutated in progressive intrahepatic cholestasis

Nazer, H, Wright, AC, Bjorkhem, I, Russell, DW, Davis, DL, Schwarz, M

J Clin Invest 2000
12679481 Molecular genetics of 3beta-hydroxy-Delta5-C27-steroid oxidoreductase deficiency in 16 patients with loss of bile acid synthesis and liver disease

Nazer, H, Gerhardt, M, Cheng, JB, Russell, DW, Jacquemin, E, Setchell, KD, Cresteil, D, Heubi, JE

J Clin Endocrinol Metab 2003
Participants
Participates
Catalyst Activity

3-beta-hydroxy-delta5-steroid dehydrogenase activity of HSD3B7 [endoplasmic reticulum membrane]

Orthologous Events
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