{"entryType":"UniProtKB reviewed (Swiss-Prot)","primaryAccession":"P15538","secondaryAccessions":["Q14095","Q4VAQ8","Q4VAQ9","Q9UML2"],"uniProtkbId":"C11B1_HUMAN","entryAudit":{"firstPublicDate":"1990-04-01","lastAnnotationUpdateDate":"2026-09-02","lastSequenceUpdateDate":"2008-11-25","entryVersion":235,"sequenceVersion":5},"annotationScore":5.0,"organism":{"scientificName":"Homo sapiens","commonName":"Human","taxonId":9606,"lineage":["Eukaryota","Metazoa","Chordata","Craniata","Vertebrata","Euteleostomi","Mammalia","Eutheria","Euarchontoglires","Primates","Haplorrhini","Catarrhini","Hominidae","Homo"]},"proteinExistence":"1: Evidence at protein level","proteinDescription":{"recommendedName":{"fullName":{"value":"Cytochrome P450 11B1, mitochondrial"},"shortNames":[{"value":"CYP11B1"}]},"alternativeNames":[{"fullName":{"value":"CYPXIB1"}},{"fullName":{"value":"Cytochrome P-450c11"},"shortNames":[{"value":"Cytochrome P450C11"}]},{"fullName":{"evidences":[{"evidenceCode":"ECO:0000303","source":"PubMed","id":"2592361"}],"value":"Steroid 11-beta-hydroxylase, CYP11B1"},"ecNumbers":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"18215163"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"23685396"}],"value":"1.14.15.4"}]}],"flag":"Precursor"},"genes":[{"geneName":{"evidences":[{"evidenceCode":"ECO:0000303","source":"PubMed","id":"18215163"},{"evidenceCode":"ECO:0000312","source":"HGNC","id":"HGNC:2591"}],"value":"CYP11B1"},"synonyms":[{"value":"S11BH"}]}],"comments":[{"texts":[{"evidences":[{"evidenceCode":"ECO:0000250","source":"UniProtKB","id":"P15393"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"12530636"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"1518866"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"1775135"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"18215163"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"23322723"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"23685396"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"29277707"},{"evidenceCode":"ECO:0000305","source":"PubMed","id":"23322723"}],"value":"A cytochrome P450 monooxygenase mainly involved in the biosynthesis of adrenal corticoids (PubMed:12530636, PubMed:1518866, PubMed:1775135, PubMed:18215163, PubMed:23322723, PubMed:23685396, PubMed:29277707). Catalyzes a variety of reactions that are essential for many species, including detoxification, defense, and the formation of endogenous chemicals like steroid hormones. Steroid 11beta, 18- and 19-hydroxylase with preferred regioselectivity at 11beta, then 18, and lastly 19 (By similarity). Catalyzes the hydroxylation of 11-deoxycortisol and 11-deoxycorticosterone (21-hydroxyprogesterone) at 11beta position, yielding cortisol or corticosterone, respectively, but cannot produce aldosterone (PubMed:12530636, PubMed:1518866, PubMed:1775135, PubMed:18215163, PubMed:23322723, PubMed:23685396, PubMed:29277707). Mechanistically, uses molecular oxygen inserting one oxygen atom into a substrate for hydroxylation and reducing the second into a water molecule. Two electrons are provided by NADPH via a two-protein mitochondrial transfer system comprising flavoprotein FDXR (adrenodoxin/ferredoxin reductase) and nonheme iron-sulfur protein FDX1 or FDX2 (adrenodoxin/ferredoxin) (PubMed:18215163, PubMed:23685396). Due to its lack of 18-oxidation activity, it is incapable of generating aldosterone (PubMed:23322723). Besides its role in the biosynthesis of mineralocorticoids and glucocorticoids, it can also participate in the androgen metabolic pathway, converting androst-4-ene-3,17-dione to 11beta-hydroxyandrost-4-ene-3,17-dione, and testosterone (17beta-hydroxy-4-androsten-3-one) to 11beta,17beta-dihydroxyandrost-4-ene-3-one (PubMed:23685396, PubMed:29277707)"}],"commentType":"FUNCTION"},{"commentType":"CATALYTIC ACTIVITY","reaction":{"name":"a steroid + 2 reduced [adrenodoxin] + O2 + 2 H(+) = an 11beta-hydroxysteroid + 2 oxidized [adrenodoxin] + H2O","reactionCrossReferences":[{"database":"Rhea","id":"RHEA:15629"},{"database":"Rhea","id":"RHEA-COMP:9998"},{"database":"Rhea","id":"RHEA-COMP:9999"},{"database":"ChEBI","id":"CHEBI:15377"},{"database":"ChEBI","id":"CHEBI:15378"},{"database":"ChEBI","id":"CHEBI:15379"},{"database":"ChEBI","id":"CHEBI:33737"},{"database":"ChEBI","id":"CHEBI:33738"},{"database":"ChEBI","id":"CHEBI:35341"},{"database":"ChEBI","id":"CHEBI:35346"}],"ecNumber":"1.14.15.4","evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"12530636"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"1518866"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"1775135"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"18215163"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"23322723"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"23685396"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"29277707"}]},"physiologicalReactions":[{"directionType":"left-to-right","reactionCrossReference":{"database":"Rhea","id":"RHEA:15630"},"evidences":[{"evidenceCode":"ECO:0000305","source":"PubMed","id":"12530636"},{"evidenceCode":"ECO:0000305","source":"PubMed","id":"1518866"},{"evidenceCode":"ECO:0000305","source":"PubMed","id":"1775135"},{"evidenceCode":"ECO:0000305","source":"PubMed","id":"18215163"},{"evidenceCode":"ECO:0000305","source":"PubMed","id":"23322723"}]}]},{"commentType":"CATALYTIC ACTIVITY","reaction":{"name":"11-deoxycortisol + 2 reduced [adrenodoxin] + O2 + 2 H(+) = cortisol + 2 oxidized [adrenodoxin] + H2O","reactionCrossReferences":[{"database":"Rhea","id":"RHEA:46100"},{"database":"Rhea","id":"RHEA-COMP:9998"},{"database":"Rhea","id":"RHEA-COMP:9999"},{"database":"ChEBI","id":"CHEBI:15377"},{"database":"ChEBI","id":"CHEBI:15378"},{"database":"ChEBI","id":"CHEBI:15379"},{"database":"ChEBI","id":"CHEBI:17650"},{"database":"ChEBI","id":"CHEBI:28324"},{"database":"ChEBI","id":"CHEBI:33737"},{"database":"ChEBI","id":"CHEBI:33738"}],"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"12530636"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"1775135"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"18215163"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"23322723"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"23685396"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"29277707"}]},"physiologicalReactions":[{"directionType":"left-to-right","reactionCrossReference":{"database":"Rhea","id":"RHEA:46101"},"evidences":[{"evidenceCode":"ECO:0000305","source":"PubMed","id":"12530636"},{"evidenceCode":"ECO:0000305","source":"PubMed","id":"1775135"},{"evidenceCode":"ECO:0000305","source":"PubMed","id":"18215163"},{"evidenceCode":"ECO:0000305","source":"PubMed","id":"23322723"}]}]},{"commentType":"CATALYTIC ACTIVITY","reaction":{"name":"21-hydroxyprogesterone + 2 reduced [adrenodoxin] + O2 + 2 H(+) = corticosterone + 2 oxidized [adrenodoxin] + H2O","reactionCrossReferences":[{"database":"Rhea","id":"RHEA:46104"},{"database":"Rhea","id":"RHEA-COMP:9998"},{"database":"Rhea","id":"RHEA-COMP:9999"},{"database":"ChEBI","id":"CHEBI:15377"},{"database":"ChEBI","id":"CHEBI:15378"},{"database":"ChEBI","id":"CHEBI:15379"},{"database":"ChEBI","id":"CHEBI:16827"},{"database":"ChEBI","id":"CHEBI:16973"},{"database":"ChEBI","id":"CHEBI:33737"},{"database":"ChEBI","id":"CHEBI:33738"}],"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"12530636"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"1518866"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"1775135"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"18215163"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"23322723"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"23685396"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"29277707"}]},"physiologicalReactions":[{"directionType":"left-to-right","reactionCrossReference":{"database":"Rhea","id":"RHEA:46105"},"evidences":[{"evidenceCode":"ECO:0000305","source":"PubMed","id":"12530636"},{"evidenceCode":"ECO:0000305","source":"PubMed","id":"1518866"},{"evidenceCode":"ECO:0000305","source":"PubMed","id":"1775135"},{"evidenceCode":"ECO:0000305","source":"PubMed","id":"18215163"},{"evidenceCode":"ECO:0000305","source":"PubMed","id":"23322723"}]}]},{"commentType":"CATALYTIC ACTIVITY","reaction":{"name":"androst-4-ene-3,17-dione + 2 reduced [adrenodoxin] + O2 + 2 H(+) = 11beta-hydroxyandrost-4-ene-3,17-dione + 2 oxidized [adrenodoxin] + H2O","reactionCrossReferences":[{"database":"Rhea","id":"RHEA:84071"},{"database":"Rhea","id":"RHEA-COMP:9998"},{"database":"Rhea","id":"RHEA-COMP:9999"},{"database":"ChEBI","id":"CHEBI:15377"},{"database":"ChEBI","id":"CHEBI:15378"},{"database":"ChEBI","id":"CHEBI:15379"},{"database":"ChEBI","id":"CHEBI:16422"},{"database":"ChEBI","id":"CHEBI:27967"},{"database":"ChEBI","id":"CHEBI:33737"},{"database":"ChEBI","id":"CHEBI:33738"}],"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"23685396"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"29277707"}]},"physiologicalReactions":[{"directionType":"left-to-right","reactionCrossReference":{"database":"Rhea","id":"RHEA:84072"},"evidences":[{"evidenceCode":"ECO:0000305","source":"PubMed","id":"23685396"}]}]},{"commentType":"CATALYTIC ACTIVITY","reaction":{"name":"testosterone + 2 reduced [adrenodoxin] + O2 + 2 H(+) = 11beta,17beta-dihydroxyandrost-4-ene-3-one + 2 oxidized [adrenodoxin] + H2O","reactionCrossReferences":[{"database":"Rhea","id":"RHEA:86455"},{"database":"Rhea","id":"RHEA-COMP:9998"},{"database":"Rhea","id":"RHEA-COMP:9999"},{"database":"ChEBI","id":"CHEBI:15377"},{"database":"ChEBI","id":"CHEBI:15378"},{"database":"ChEBI","id":"CHEBI:15379"},{"database":"ChEBI","id":"CHEBI:17347"},{"database":"ChEBI","id":"CHEBI:33737"},{"database":"ChEBI","id":"CHEBI:33738"},{"database":"ChEBI","id":"CHEBI:81481"}],"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"23685396"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"29277707"}]},"physiologicalReactions":[{"directionType":"left-to-right","reactionCrossReference":{"database":"Rhea","id":"RHEA:86456"},"evidences":[{"evidenceCode":"ECO:0000305","source":"PubMed","id":"23685396"}]}]},{"commentType":"COFACTOR","cofactors":[{"name":"heme","evidences":[{"evidenceCode":"ECO:0000250","source":"UniProtKB","id":"P19099"}],"cofactorCrossReference":{"database":"ChEBI","id":"CHEBI:30413"}}]},{"commentType":"BIOPHYSICOCHEMICAL PROPERTIES","kineticParameters":{"michaelisConstants":[{"constant":338.4,"unit":"uM","substrate":"11-deoxycortisol","evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"18215163"}]},{"constant":179.5,"unit":"uM","substrate":"21-hydroxyprogesterone","evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"18215163"}]}],"note":{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"18215163"}],"value":"kcat is 1.67 sec(-1) with 11-deoxycortisol as substrate. kcat is 0.85 sec(-1) with 21-hydroxyprogesterone as substrate."}]}}},{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"18215163"}],"value":"Steroid biosynthesis; glucocorticoid biosynthesis"}],"commentType":"PATHWAY"},{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"18215163"}],"value":"Steroid hormone biosynthesis"}],"commentType":"PATHWAY"},{"commentType":"SUBCELLULAR LOCATION","subcellularLocations":[{"location":{"evidences":[{"evidenceCode":"ECO:0000250","source":"UniProtKB","id":"P14137"}],"value":"Mitochondrion inner membrane","id":"SL-0168"},"topology":{"evidences":[{"evidenceCode":"ECO:0000250","source":"UniProtKB","id":"P14137"}],"value":"Peripheral membrane protein","id":"SL-9903"}}]},{"commentType":"ALTERNATIVE PRODUCTS","events":["Alternative splicing"],"isoforms":[{"name":{"value":"1"},"isoformIds":["P15538-1"],"isoformSequenceStatus":"Displayed"},{"name":{"value":"2"},"isoformIds":["P15538-2"],"sequenceIds":["VSP_043308"],"isoformSequenceStatus":"Described"}]},{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"20200334"}],"value":"Expressed in the zona fasciculata/reticularis of the adrenal cortex"}],"commentType":"TISSUE SPECIFICITY"},{"commentType":"DISEASE","disease":{"diseaseId":"Adrenal hyperplasia 4","diseaseAccession":"DI-00044","acronym":"AH4","description":"A form of congenital adrenal hyperplasia, a common recessive disease due to defective synthesis of cortisol. Congenital adrenal hyperplasia is characterized by androgen excess leading to ambiguous genitalia in affected females, rapid somatic growth during childhood in both sexes with premature closure of the epiphyses and short adult stature. Four clinical types: 'salt wasting' (SW, the most severe type), 'simple virilizing' (SV, less severely affected patients), with normal aldosterone biosynthesis, 'non-classic form' or late-onset (NC or LOAH) and 'cryptic' (asymptomatic).","diseaseCrossReference":{"database":"MIM","id":"202010"},"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"16046588"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"20089618"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"2022736"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"20331679"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"20947076"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"23940125"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"24022297"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"24536089"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"24987415"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"26053152"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"26476331"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"9302260"}]},"note":{"texts":[{"value":"The disease is caused by variants affecting the gene represented in this entry"}]}},{"commentType":"DISEASE","disease":{"diseaseId":"Hyperaldosteronism, familial, 1","diseaseAccession":"DI-02693","acronym":"HALD1","description":"A disorder characterized by hypertension, variable hyperaldosteronism, and abnormal adrenal steroid production, including 18-oxocortisol and 18-hydroxycortisol. There is significant phenotypic heterogeneity, and some individuals never develop hypertension.","diseaseCrossReference":{"database":"MIM","id":"103900"}},"note":{"texts":[{"value":"The disease is caused by variants affecting the gene represented in this entry. The molecular defect causing hyperaldosteronism familial 1 is an anti-Lepore-type fusion of the CYP11B1 and CYP11B2 genes. The hybrid gene has the promoting part of CYP11B1, ACTH-sensitive, and the coding part of CYP11B2"}]}},{"texts":[{"evidences":[{"evidenceCode":"ECO:0000305"}],"value":"Belongs to the cytochrome P450 family"}],"commentType":"SIMILARITY"}],"features":[{"type":"Transit peptide","location":{"start":{"value":1,"modifier":"EXACT"},"end":{"value":24,"modifier":"EXACT"}},"description":"Mitochondrion"},{"type":"Chain","location":{"start":{"value":25,"modifier":"EXACT"},"end":{"value":503,"modifier":"EXACT"}},"description":"Cytochrome P450 11B1, mitochondrial","featureId":"PRO_0000003596"},{"type":"Binding site","location":{"start":{"value":450,"modifier":"EXACT"},"end":{"value":450,"modifier":"EXACT"}},"description":"axial binding 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activity","evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"20089618"}],"featureId":"VAR_074502","alternativeSequence":{"originalSequence":"F","alternativeSequences":["L"]}},{"type":"Natural variant","location":{"start":{"value":141,"modifier":"EXACT"},"end":{"value":141,"modifier":"EXACT"}},"description":"in AH4; uncertain significance; dbSNP:rs267601810","featureCrossReferences":[{"database":"dbSNP","id":"rs267601810"}],"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"24987415"}],"featureId":"VAR_075553","alternativeSequence":{"originalSequence":"R","alternativeSequences":["Q"]}},{"type":"Natural variant","location":{"start":{"value":143,"modifier":"EXACT"},"end":{"value":143,"modifier":"EXACT"}},"description":"in AH4; non-classic; highly decreases steroid 11-beta-hydroxylase activity; 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