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Literature summary extracted from

  • Voegeli, I.; Jung, H.H.; Dick, B.; Erickson, S.K.; Escher, R.; Funder, J.W.; Frey, F.J.; Escher, G.
    Evidence for a role of sterol 27-hydroxylase in glucocorticoid metabolism in vivo (2013), J. Endocrinol., 219, 119-129.
    View publication on PubMed

Protein Variants

EC Number Protein Variants Comment Organism
1.14.15.15 additional information construction of enzyme knockout null mutant mice Mus musculus

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
1.14.15.15 mitochondrion
-
Homo sapiens 5739
-
1.14.15.15 mitochondrion
-
Mus musculus 5739
-

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
1.14.15.15 cholesterol + reduced adrenodoxin + O2 Homo sapiens
-
27-hydroxycholesterol + oxidized adrenodoxin + H2O
-
?
1.14.15.15 cholesterol + reduced adrenodoxin + O2 Mus musculus
-
27-hydroxycholesterol + oxidized adrenodoxin + H2O
-
?

Organism

EC Number Organism UniProt Comment Textmining
1.14.15.15 Homo sapiens Q02318 gene CYP27A1
-
1.14.15.15 Mus musculus Q9DBG1 gene CYP27A1
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
1.14.15.15 liver
-
Homo sapiens
-
1.14.15.15 liver
-
Mus musculus
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.14.15.15 cholesterol + reduced adrenodoxin + O2
-
Homo sapiens 27-hydroxycholesterol + oxidized adrenodoxin + H2O
-
?
1.14.15.15 cholesterol + reduced adrenodoxin + O2
-
Mus musculus 27-hydroxycholesterol + oxidized adrenodoxin + H2O
-
?

Synonyms

EC Number Synonyms Comment Organism
1.14.15.15 CYP27A1
-
Homo sapiens
1.14.15.15 CYP27A1
-
Mus musculus
1.14.15.15 sterol 27-hydroxylase
-
Homo sapiens
1.14.15.15 sterol 27-hydroxylase
-
Mus musculus

Cofactor

EC Number Cofactor Comment Organism Structure
1.14.15.15 adrenodoxin
-
Homo sapiens
1.14.15.15 adrenodoxin
-
Mus musculus
1.14.15.15 NADPH dependent on Homo sapiens
1.14.15.15 NADPH dependent on Mus musculus

General Information

EC Number General Information Comment Organism
1.14.15.15 malfunction CYP27A1 deficiency may upregulate the activity of 11beta-hydroxysteroid dehydrogenase 1, and downregulate the activity of 11beta-hydroxysteroid dehydrogenase 2. In a patient with cerebrotendinous xanthomatosis carrying a loss-of-function mutation in CYP27A1, the plasma concentrations of 27-hydroxycholesterol are dramatically reduced, with enhanced HSD11B1 and diminished HSD11B2 activities Homo sapiens
1.14.15.15 malfunction in Cyp27a1 knockout mice, the plasma concentrations of 27-hydroxycholesterol are undetectable. In the liver of the mutant mice, the increase in concentrations of active glucocorticoids is due to increased liver weight as a consequence of Cyp27a1 deficiency Mus musculus
1.14.15.15 physiological function in the liver, CYP27A1 catalyses the first step of the alternative pathway of bile acid biosynthesis and intermediate reactions in the classical pathway initiated by CYP7A1. In extrahepatic tissues, CYP27A1 plays a role in reverse cholesterol transport because its product 27-hydroxycholesterol is removed and carried to the liver, where it is converted to bile acids. 27-Hydroxycholesterol is a key regulator of cholesterol homeostasis. Sterol 27-hydroxylase (CYP27A1) catalyses the first step in the alternative pathway of bile acid synthesis by hydroxylating cholesterol to 27-hydroxycholesterol, which is a natural ligand for liver X receptor. In vitro agonist treatment of liver X receptor downregulates the activity of 11beta-hydroxysteroid dehydrogenase 1, HSD11B1, that is involved in regulation of intracellular availability of glucocorticoids Homo sapiens
1.14.15.15 physiological function in the liver, CYP27A1 catalyses the first step of the alternative pathway of bile acid biosynthesis and intermediate reactions in the classical pathway initiated by CYP7A1. In extrahepatic tissues, CYP27A1 plays a role in reverse cholesterol transport because its product 27-hydroxycholesterol is removed and carried to the liver, where it is converted to bile acids. 27-Hydroxycholesterol is a key regulator of cholesterol homeostasis. Sterol 27-hydroxylase (CYP27A1) catalyses the first step in the alternative pathway of bile acid synthesis by hydroxylating cholesterol to 27-hydroxycholesterol, which is a natural ligand for liver X receptor. In vitro agonist treatment of liver X receptor downregulates the activity of 11beta-hydroxysteroid dehydrogenase 1, HSD11B1, that is involved in regulation of intracellular availability of glucocorticoids Mus musculus