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Literature summary for 1.14.15.15 extracted from
- Evidence for a role of sterol 27-hydroxylase in glucocorticoid metabolism in vivo (2013), J. Endocrinol., 219, 119-129.
Protein Variants
| Protein Variants | Comment | Organism |
|---|---|---|
| additional information | construction of enzyme knockout null mutant mice | Mus musculus |
Localization
| Localization | Comment | Organism | GeneOntology No. | Textmining |
|---|---|---|---|---|
| mitochondrion | - |
Homo sapiens | 5739 | - |
| mitochondrion | - |
Mus musculus | 5739 | - |
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| cholesterol + reduced adrenodoxin + O2 | Homo sapiens | - |
27-hydroxycholesterol + oxidized adrenodoxin + H2O | - |
? |  |
| cholesterol + reduced adrenodoxin + O2 | Mus musculus | - |
27-hydroxycholesterol + oxidized adrenodoxin + H2O | - |
? | |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | Q02318 | gene CYP27A1 | - |
| Mus musculus | Q9DBG1 | gene CYP27A1 | - |
Source Tissue
| Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|
| liver | - |
Homo sapiens | - |
| liver | - |
Mus musculus | - |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| cholesterol + reduced adrenodoxin + O2 | - |
Homo sapiens | 27-hydroxycholesterol + oxidized adrenodoxin + H2O | - |
? | |
| cholesterol + reduced adrenodoxin + O2 | - |
Mus musculus | 27-hydroxycholesterol + oxidized adrenodoxin + H2O | - |
? | |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| CYP27A1 | - |
Homo sapiens |
| CYP27A1 | - |
Mus musculus |
| sterol 27-hydroxylase | - |
Homo sapiens |
| sterol 27-hydroxylase | - |
Mus musculus |
Cofactor
| Cofactor | Comment | Organism | Structure |
|---|---|---|---|
| adrenodoxin | - |
Homo sapiens | |
| adrenodoxin | - |
Mus musculus | |
| NADPH | dependent on | Homo sapiens | |
| NADPH | dependent on | Mus musculus | |
General Information
| General Information | Comment | Organism |
|---|---|---|
| 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 |
| 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 |
| 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 |
| 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 |
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