Free for academic non-profit institutions. Other users need a Commercial license

Aliases for CYP11B1 Gene

Aliases for CYP11B1 Gene

  • Cytochrome P450, Family 11, Subfamily B, Polypeptide 1 2 3
  • Cytochrome P450, Subfamily XIB (Steroid 11-Beta-Hydroxylase), Polypeptide 1 2 3
  • Steroid 11-Beta-Monooxygenase 2 3
  • Steroid 11-Beta-Hydroxylase 3 4
  • Cytochrome P-450c11 3 4
  • Cytochrome P450C11 3 4
  • EC 4 63
  • CYPXIB1 3 4
  • Corticosteroid Methyl-Oxidase II (CMO II)) 6
  • Cytochrome P450 11B1, Mitochondrial 3
  • Cytochrome P450 XIB1 3
  • EC 1.14.15 63
  • P450C11 3
  • CYP11B 3
  • S11BH 4
  • CPN1 3
  • FHI 3

External Ids for CYP11B1 Gene

Previous HGNC Symbols for CYP11B1 Gene

  • CYP11B

Previous GeneCards Identifiers for CYP11B1 Gene

  • GC08M142258
  • GC08M144187
  • GC08M143758
  • GC08M144016
  • GC08M143950
  • GC08M139206

Summaries for CYP11B1 Gene

Entrez Gene Summary for CYP11B1 Gene

  • This gene encodes a member of the cytochrome P450 superfamily of enzymes. The cytochrome P450 proteins are monooxygenases which catalyze many reactions involved in drug metabolism and synthesis of cholesterol, steroids and other lipids. This protein localizes to the mitochondrial inner membrane and is involved in the conversion of progesterone to cortisol in the adrenal cortex. Mutations in this gene cause congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency. Transcript variants encoding different isoforms have been noted for this gene. [provided by RefSeq, Jul 2008]

GeneCards Summary for CYP11B1 Gene

CYP11B1 (Cytochrome P450, Family 11, Subfamily B, Polypeptide 1) is a Protein Coding gene. Diseases associated with CYP11B1 include adrenal hyperplasia, congenital, due to 11-beta-hydroxylase deficiency and aldosteronism, glucocorticoid-remediable. Among its related pathways are Metabolism and Metabolism. GO annotations related to this gene include electron carrier activity and heme binding. An important paralog of this gene is CYP11A1.

UniProtKB/Swiss-Prot for CYP11B1 Gene

  • Has steroid 11-beta-hydroxylase activity. In addition to this activity, the 18 or 19-hydroxylation of steroids and the aromatization of androstendione to estrone have also been ascribed to cytochrome P450 XIB

Tocris Summary for CYP11B1 Gene

  • Cytochrome P450 (CYP450) enzymes are a diverse group of catalysts that contains 57 members in humans. CYPs are usually membrane-bound and are localized to the inner mitochondrial or endoplasmic reticular membrane. CYPs have oxygenase activity and commonly catalyze redox reactions, involving the oxidation of the substrate and reduction of water. This group of enzymes contain a heme ion within the active site, which is essential for catalytic activity. CYPs have been found in all organisms tested and are ubiquitously expressed. They are found at high levels in the liver, where they have an important role in metabolism of drugs and endogenous toxic compounds (for example bilirubin). Most CYPs can metabolize numerous substrates and this accounts for their major role in drug interactions. CYPs also have functions in steroid hormone synthesis, cholesterol synthesis and vitamin D metabolism.

Gene Wiki entry for CYP11B1 Gene

No data available for PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for CYP11B1 Gene

Genomics for CYP11B1 Gene

Regulatory Elements for CYP11B1 Gene

Genomic Location for CYP11B1 Gene

142,872,356 bp from pter
142,879,846 bp from pter
7,491 bases
Minus strand

Genomic View for CYP11B1 Gene

UCSC Golden Path with GeneCards custom track
Cytogenetic band:
Genomic Location for CYP11B1 Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for CYP11B1 Gene

Proteins for CYP11B1 Gene

  • Protein details for CYP11B1 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Cytochrome P450 11B1, mitochondrial
    Protein Accession:
    Secondary Accessions:
    • Q14095
    • Q4VAQ8
    • Q4VAQ9
    • Q9UML2

    Protein attributes for CYP11B1 Gene

    503 amino acids
    Molecular mass:
    57573 Da
    Name=heme; Xref=ChEBI:CHEBI:30413;
    Quaternary structure:
    No Data Available

    Alternative splice isoforms for CYP11B1 Gene


neXtProt entry for CYP11B1 Gene

Proteomics data for CYP11B1 Gene at MOPED

Selected DME Specific Peptides for CYP11B1 Gene

Post-translational modifications for CYP11B1 Gene

  • Modification sites at PhosphoSitePlus
  • Modification sites at neXtProt

Other Protein References for CYP11B1 Gene

ENSEMBL proteins:
Reactome Protein details:
REFSEQ proteins:

Antibody Products

Domains for CYP11B1 Gene

Gene Families for CYP11B1 Gene

Protein Domains for CYP11B1 Gene

Graphical View of Domain Structure for InterPro Entry



  • Belongs to the cytochrome P450 family.
  • Belongs to the cytochrome P450 family.
genes like me logo Genes that share domains with CYP11B1: view

Function for CYP11B1 Gene

Molecular function for CYP11B1 Gene

GENATLAS Biochemistry:
cytochrome p450,subfamily XIB,polypeptide 1,steroid 11 beta-hydroxylase,mitochondrial,involved in glucocorticoid biosynthesis,stimulated by corticotropin,expressed in zona fasciculata of the adrenal cortex
UniProtKB/Swiss-Prot CatalyticActivity:
A steroid + 2 reduced adrenodoxin + O(2) + 2 H(+) = an 11-beta- hydroxysteroid + 2 oxidized adrenodoxin + H(2)O
UniProtKB/Swiss-Prot Function:
Has steroid 11-beta-hydroxylase activity. In addition to this activity, the 18 or 19-hydroxylation of steroids and the aromatization of androstendione to estrone have also been ascribed to cytochrome P450 XIB

Enzyme Numbers (IUBMB) for CYP11B1 Gene

Gene Ontology (GO) - Molecular Function for CYP11B1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0004497 monooxygenase activity --
GO:0004507 steroid 11-beta-monooxygenase activity IMP 2022736
GO:0005506 iron ion binding IEA --
GO:0016705 oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen --
GO:0020037 heme binding IC 2256920
genes like me logo Genes that share ontologies with CYP11B1: view
genes like me logo Genes that share phenotypes with CYP11B1: view

Animal Model Products

CRISPR Products

miRNA Products

Inhibitory RNA Products

  • Predesigned siRNA for gene silencing in human,mouse,rat for CYP11B1

In Situ Assay Products

Flow Cytometry Products

No data available for Animal Models , miRNA , Transcription Factor Targets and HOMER Transcription for CYP11B1 Gene

Localization for CYP11B1 Gene

Subcellular locations from UniProtKB/Swiss-Prot for CYP11B1 Gene

Mitochondrion membrane.

Subcellular locations from

Jensen Localization Image for CYP11B1 Gene COMPARTMENTS Subcellular localization image for CYP11B1 gene
Compartment Confidence
mitochondrion 5
cytosol 2
nucleus 2
peroxisome 2

Gene Ontology (GO) - Cellular Components for CYP11B1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005739 mitochondrion IDA 2256920
GO:0005743 mitochondrial inner membrane TAS 15026188
genes like me logo Genes that share ontologies with CYP11B1: view

Pathways for CYP11B1 Gene

genes like me logo Genes that share pathways with CYP11B1: view

PCR Array Products

  • Pathway & Disease-focused RT² Profiler PCR Arrays

Gene Ontology (GO) - Biological Process for CYP11B1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0006700 C21-steroid hormone biosynthetic process IDA 2256920
GO:0006704 glucocorticoid biosynthetic process TAS --
GO:0006705 mineralocorticoid biosynthetic process --
GO:0006805 xenobiotic metabolic process TAS --
GO:0006955 immune response TAS 12452430
genes like me logo Genes that share ontologies with CYP11B1: view

Drugs for CYP11B1 Gene

(14) Drugbank Compounds for CYP11B1 Gene

Compound Status Synonyms Cas Number PubChem ID Type Actions PubMed IDs
  • Extina
65277-42-1 Enzyme inhibitor approved, investigational
  • 2-Methoxy-4-amino-5-chloro-N,N-(dimethylaminoethyl)benzamide
364-62-5 4168 Enzyme inhibitor approved, investigational
  • 1-(2,4-Dichloro-beta-((2,4-dichlorobenzyl)oxy)phenethyl)imidazole
22916-47-8 4189 Enzyme inhibitor approved, investigational
  • 1-Cyano-2-methyl-3-(2-(((5-methyl-4-imidazolyl)methyl)thio)ethyl)guanidine
51481-61-9 2756 Enzyme inhibitor approved
  • 1-((2-Chlorophenyl)diphenylmethyl)-1H-imidazole
23593-75-1 2812 Enzyme inhibitor approved

(3) Tocris Compounds for CYP11B1 Gene

Compound Action Cas Number
IOX 2 Potent, selective HIF-1alpha prolyl hydroxylase-2 (PHD2) inhibitor [931398-72-0]
Metyrapone 11-beta hydroxylase inhibitor [54-36-4]
Ro 61-8048 Potent kynurenine 3-hydroxylase inhibitor [199666-03-0]

(27) HMDB Compounds for CYP11B1 Gene

Compound Synonyms Cas Number PubMed IDs
  • 11beta,17alpha,21-Trihydroxypreg-nenolone
  • 11beta-Hydroxyandrost-4-ene-3,17-dione
  • (11beta)-11-hydroxypregn-4-ene-3,20-dione
  • 17alpha,21-Dihydroxypreg-nenolone
  • 17-alpha-Hydroxyprogesterone

(38) Novoseek inferred chemical compound relationships for CYP11B1 Gene

Compound -log(P) Hits PubMed IDs
11 deoxycortisol 91.5 12
18-oxocortisol 91.5 5
18-hydroxycorticosterone 89.1 3
18-hydroxycortisol 88 3
doca 86.2 8
genes like me logo Genes that share compounds with CYP11B1: view

Transcripts for CYP11B1 Gene

Unigene Clusters for CYP11B1 Gene

Cytochrome P450, family 11, subfamily B, polypeptide 1:
Representative Sequences:

CRISPR Products

miRNA Products

Inhibitory RNA Products

  • Predesigned siRNA for gene silencing in human,mouse,rat for CYP11B1

Flow Cytometry Products

Alternative Splicing Database (ASD) splice patterns (SP) for CYP11B1 Gene

No ASD Table

Relevant External Links for CYP11B1 Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for CYP11B1 Gene

mRNA expression in normal human tissues for CYP11B1 Gene

mRNA differential expression in normal tissues according to GTEx for CYP11B1 Gene

This gene is overexpressed in Adrenal Gland (52.7).

Protein differential expression in normal tissues for CYP11B1 Gene

This gene is overexpressed in Adrenal (69.0).

Integrated Proteomics: protein expression from ProteomicsDB, PaxDb, and MOPED for CYP11B1 Gene

SOURCE GeneReport for Unigene cluster for CYP11B1 Gene Hs.184927

genes like me logo Genes that share expressions with CYP11B1: view

Expression partners for CYP11B1 Gene

* - Elite partner

Primer Products

In Situ Assay Products

No data available for mRNA expression in embryonic tissues and stem cells from LifeMap Discovery and mRNA Expression by UniProt/SwissProt for CYP11B1 Gene

Orthologs for CYP11B1 Gene

This gene was present in the common ancestor of animals.

Orthologs for CYP11B1 Gene

Organism Taxonomy Gene Similarity Type Details
(Mus musculus)
Mammalia Cyp11b2 35
  • 74.87 (n)
  • 68.2 (a)
Cyp11b1 16
Cyp11b1 36
  • 64 (a)
Cyp11b2 36
  • 68 (a)
(Pan troglodytes)
Mammalia LOC464437 35
  • 98.34 (n)
  • 98.46 (a)
CYP11B1 36
  • 90 (a)
(Rattus norvegicus)
Mammalia Cyp11b2 35
  • 75.2 (n)
  • 68.6 (a)
(Bos Taurus)
Mammalia -- 36
  • 72 (a)
-- 36
  • 69 (a)
(Canis familiaris)
Mammalia -- 36
  • 72 (a)
(Monodelphis domestica)
Mammalia -- 36
  • 52 (a)
(Ornithorhynchus anatinus)
Mammalia -- 36
  • 48 (a)
(Anolis carolinensis)
Reptilia -- 36
  • 50 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia LOC101731070 35
  • 57.52 (n)
  • 51.36 (a)
(Danio rerio)
Actinopterygii cyp11c1 35
  • 55.27 (n)
  • 43.87 (a)
cyp11c1 36
  • 32 (a)
fruit fly
(Drosophila melanogaster)
Insecta Cyp12c1 37
  • 29 (a)
Cyp12a4 36
  • 22 (a)
Cyp12a5 36
  • 20 (a)
Cyp12b2 36
  • 21 (a)
Cyp12c1 36
  • 23 (a)
Cyp12d1-d 36
  • 20 (a)
Cyp12d1-p 36
  • 20 (a)
Cyp12e1 36
  • 21 (a)
Cyp301a1 36
  • 18 (a)
Cyp49a1 36
  • 19 (a)
dib 36
  • 24 (a)
shd 36
  • 21 (a)
(Caenorhabditis elegans)
Secernentea ccp-44 37
  • 26 (a)
cyp-44A1 36
  • 24 (a)
sea squirt
(Ciona savignyi)
Ascidiacea -- 36
  • 20 (a)
Species with no ortholog for CYP11B1:
  • A. gosspyii yeast (Ashbya gossypii)
  • Actinobacteria (Mycobacterium tuberculosis)
  • African clawed frog (Xenopus laevis)
  • African malaria mosquito (Anopheles gambiae)
  • Alicante grape (Vitis vinifera)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • baker's yeast (Saccharomyces cerevisiae)
  • barley (Hordeum vulgare)
  • beta proteobacteria (Neisseria meningitidis)
  • bread mold (Neurospora crassa)
  • chicken (Gallus gallus)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • corn (Zea mays)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • fission yeast (Schizosaccharomyces pombe)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • K. lactis yeast (Kluyveromyces lactis)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • rainbow trout (Oncorhynchus mykiss)
  • rice (Oryza sativa)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • soybean (Glycine max)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • thale cress (Arabidopsis thaliana)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • wheat (Triticum aestivum)

Evolution for CYP11B1 Gene

Gene Tree for CYP11B1 (if available)
Gene Tree for CYP11B1 (if available)

Paralogs for CYP11B1 Gene

Paralogs for CYP11B1 Gene Pseudogenes for CYP11B1 Gene

genes like me logo Genes that share paralogs with CYP11B1: view

Variants for CYP11B1 Gene

Sequence variations from dbSNP and Humsavar for CYP11B1 Gene

SNP ID Clin Chr 08 pos Sequence Context AA Info Type MAF
rs370725779 -- 142,873,516(+) AGTCA(A/G)TAAAA utr-variant-3-prime, downstream-variant-500B
rs370757808 -- 142,875,304(+) CCACA(A/G)GGTAG reference, missense
rs370772088 -- 142,874,924(+) ACCCC(A/G)CCCAG intron-variant
rs370724349 -- 142,879,198(+) GGGCC(A/G)AGCAC intron-variant
rs370713534 -- 142,876,408(+) GGGAG(C/T)GGGAG intron-variant

Structural Variations from Database of Genomic Variants (DGV) for CYP11B1 Gene

Variant ID Type Subtype PubMed ID
esv2661137 CNV Deletion 23128226
nsv891647 CNV Loss 21882294
nsv520940 CNV Gain 19592680
nsv482123 CNV Gain 20164927
nsv470246 CNV Loss 18288195
esv2737938 CNV Deletion 23290073
esv991235 CNV Deletion 20482838
nsv436586 CNV Deletion 17901297
nsv891648 CNV Loss 21882294
esv2673440 CNV Deletion 23128226

Relevant External Links for CYP11B1 Gene

HapMap Linkage Disequilibrium report
Human Gene Mutation Database (HGMD)

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for CYP11B1 Gene

Disorders for CYP11B1 Gene

(2) OMIM Diseases for CYP11B1 Gene (610613)


  • Adrenal hyperplasia 4 (AH4) [MIM:202010]: 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). {ECO:0000269 PubMed:16046588, ECO:0000269 PubMed:2022736, ECO:0000269 PubMed:20331679, ECO:0000269 PubMed:20947076, ECO:0000269 PubMed:9302260}. Note=The disease is caused by mutations affecting the gene represented in this entry.
  • Familial hyperaldosteronism 1 (FH1) [MIM:103900]: 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. Note=The disease is caused by mutations 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.

(20) Novoseek inferred disease relationships for CYP11B1 Gene

Disease -log(P) Hits PubMed IDs
glucocorticoid-remediable aldosteronism 95.5 20
adrenal hyperplasia congenital 90.4 39
hyperaldosteronism 89.1 18
aldosteronism primary 85.5 9
adrenal hyperplasia 78.3 3

Relevant External Links for CYP11B1

Genetic Association Database (GAD)
Human Genome Epidemiology (HuGE) Navigator
genes like me logo Genes that share disorders with CYP11B1: view

No data available for Genatlas for CYP11B1 Gene

Publications for CYP11B1 Gene

  1. Role of steroid 11 beta-hydroxylase and steroid 18-hydroxylase in the biosynthesis of glucocorticoids and mineralocorticoids in humans. (PMID: 1741400) Kawamoto T. … Shuzuta Y. (Proc. Natl. Acad. Sci. U.S.A. 1992) 3 4 23
  2. Hereditary hypertension caused by chimaeric gene duplications and ectopic expression of aldosterone synthase. (PMID: 1303253) Lifton R.P. … Laidlaw J.C. (Nat. Genet. 1992) 2 3 23
  3. CYP11B1 mutations causing non-classic adrenal hyperplasia due to 11 beta-hydroxylase deficiency. (PMID: 9302260) Joehrer K. … White P.C. (Hum. Mol. Genet. 1997) 3 4 23
  4. Mutation analysis of CYP11B1 and CYP11B2 in patients with increased 18-hydroxycortisol production. (PMID: 15062555) Nicod J. … Ferrari P. (Mol. Cell. Endocrinol. 2004) 3 23 48
  5. Mutations in CYP11B1 and congenital adrenal hyperplasia in Moroccan Jews. (PMID: 16030166) Paperna T. … Hochberg Z. (J. Clin. Endocrinol. Metab. 2005) 3 23 48

Products for CYP11B1 Gene

Sources for CYP11B1 Gene

Back to Top