MetaCyc Enzyme: 3β-hydroxy-Δ5-C27 steroid oxidoreductase

Gene: Hsd3b7 Accession Number: G-10909 (MetaCyc)

Species: Mus musculus

The subunit structure of this enzyme has not been determined.

The Hsd3b7 gene cloned from mouse liver was used to identify the corresponding human gene. Mutations in the human gene encoding this enzyme can lead to neonatal liver failure [Schwarz00] and reviewed in [Russell03].

The native enzyme has been purified from rabbit liver [Wikvall81] and pig liver [Furster96].

Predicted protein sequences from human, mouse and rat showed 86-94% sequence identity [Schwarz00].

The tissue distribution of mRNA for mouse and rat suggested liver-specific expression. However, the human mRNA was present in many tissues [Schwarz00].

Locations: endoplasmic reticulum membrane

Map Position: [134,944,123 -> 134,947,316]

Unification Links: Entrez-gene:101502

Gene-Reaction Schematic: ?

Gene-Reaction Schematic

GO Terms:

Cellular Component: GO:0005789 - endoplasmic reticulum membrane [Schwarz00]

Created 14-Oct-2008 by Fulcher CA , SRI International

Enzymatic reaction of: cholest-5-ene-3β,7α-diol 3β-dehydrogenase (3β-hydroxy-Δ5-C27 steroid oxidoreductase)

Synonyms: cholest-5-ene-3β,7α-diol:NAD+ 3-oxidoreductase

EC Number:

7α-hydroxycholesterol + NAD+ <=> 7α-hydroxycholest-4-en-3-one + NADH + H+

The reaction direction shown, that is, A + B ↔ C + D versus C + D ↔ A + B, is in accordance with the Enzyme Commission system.

The reaction is favored in the direction shown.

In Pathways: bile acid biosynthesis, neutral pathway

The recombinant mouse enzyme was transfected into HEK 293 cells and assayed for C27-3β-hydroxy-steroid dehydrogenase activity. It was only active against 7α-hydroxylated sterol substrates. Due to lack of substrate availability, the 7α-hydroxylated forms of 24-, 25- and 27-hydroxycholesterol were used as substrates [Schwarz00].


Furster96: Furster C, Zhang J, Toll A (1996). "Purification of a 3beta-hydroxy-delta5-C27-steroid dehydrogenase from pig liver microsomes active in major and alternative pathways of bile acid biosynthesis." J Biol Chem 271(34);20903-7. PMID: 8702847

Russell03: Russell DW (2003). "The enzymes, regulation, and genetics of bile acid synthesis." Annu Rev Biochem 72;137-74. PMID: 12543708

Schwarz00: Schwarz M, Wright AC, Davis DL, Nazer H, Bjorkhem I, Russell DW (2000). "The bile acid synthetic gene 3beta-hydroxy-Delta(5)-C(27)-steroid oxidoreductase is mutated in progressive intrahepatic cholestasis." J Clin Invest 106(9);1175-84. PMID: 11067870

Wikvall81: Wikvall K (1981). "Purification and properties of a 3 beta-hydroxy-delta 5-C27-steroid oxidoreductase from rabbit liver microsomes." J Biol Chem 256(7);3376-80. PMID: 6937465

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Please cite the following article in publications resulting from the use of MetaCyc: Caspi et al, Nucleic Acids Research 42:D459-D471 2014
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