1.14.14.29: 25/26-hydroxycholesterol 7alpha-hydroxylase

This is an abbreviated version!
For detailed information about 25/26-hydroxycholesterol 7alpha-hydroxylase, go to the full flat file.

Word Map on EC 1.14.14.29

Reaction

(25R)-cholest-5-ene-3beta,26-diol
+
[reduced NADPH-hemoprotein reductase]
+
O2
=
(25R)-cholest-5-ene-3beta,7alpha,26-triol
+
[oxidized NADPH-hemoprotein reductase]
+
H2O

Synonyms

25-hydroxycholesterol 7alpha-hydroxylase, 25-hydroxycholesterol 7alpha-monooxygenase, 25-hydroxycholesterol-7alpha-hydroxylase, cholesterol 25-hydroxylase, Cyp39a1, CYP7B, CYP7B1, CYP7B1 oxysterol 7alpha-hydroxylase, CYPB1, cytochome P450 7B1, cytochrome P-450 oxysterol 7-alpha-hydroxylase, oxysterol 7 alpha-hydroxylase, oxysterol 7alpha-hydroxylase, oxysterol-7alpha-hydroxylase, Steroid hydroxylase

ECTree

     1 Oxidoreductases
         1.14 Acting on paired donors, with incorporation or reduction of molecular oxygen
             1.14.14 With reduced flavin or flavoprotein as one donor, and incorporation of one atom of oxygen into the other donor
                1.14.14.29 25/26-hydroxycholesterol 7alpha-hydroxylase

General Information

General Information on EC 1.14.14.29 - 25/26-hydroxycholesterol 7alpha-hydroxylase

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GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
metabolism
CYP7B1 is an enzyme expressed in many human tissues and implicated in cholesterol metabolism. In the liver, this protein is part of the alternate/acidic pathway for primary bile acid production while in brain, CYP7B1 provides the primary metabolic route for cholesterol derivatives dehydroepiandrosterone and related hydroxysteroids via 7alpha-hydroxylation
physiological function
additional information
spastic paraplegia type 5, SPG5, is caused by mutations in CYP7B1, a gene encoding the cytochrome P-450 oxysterol 7-alpha-hydroxylase, CYP7B1, an enzyme implicated in cholesterol metabolism. Mutations in CYP7B1 are found in both pure and complicated forms of the disease, clinical phenotypes, overview