Steroid 11β-hydroxylase

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AliasesCYP11B1, CPN1, CYP11B, FHI, P450C11, cytochrome P450 family 11 subfamily B member 1
External IDsOMIM: 610613 MGI: 88584 HomoloGene: 128035 GeneCards: CYP11B1
EC number1.14.15.4
Gene location (Human)
Chromosome 8 (human)
Chr.Chromosome 8 (human)[1]
Chromosome 8 (human)
Genomic location for CYP11B1
Genomic location for CYP11B1
Band8q24.3Start142,872,354 bp[1]
End142,879,846 bp[1]
RNA expression pattern
PBB GE CYP11B1 214610 at fs.png
More reference expression data
RefSeq (mRNA)



RefSeq (protein)



Location (UCSC)Chr 8: 142.87 – 142.88 MbChr 15: 74.85 – 74.86 Mb
PubMed search[3][4]
View/Edit HumanView/Edit Mouse

Steroid 11β-hydroxylase is a steroid hydroxylase found in the zona glomerulosa and zona fasciculata. Named officially the cytochrome P450 11B1, mitochondrial, it is a protein that in humans is encoded by the CYP11B1 gene.[5][6]

This gene encodes a member of the cytochrome P450 superfamily of enzymes. The cytochrome P450 proteins are monooxygenases that 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 11-deoxycortisol to cortisol in the adrenal cortex. Transcript variants encoding different isoforms have been noted for this gene.[6]

It is reversibly inhibited by etomidate [7][8] and metyrapone.


steroid 11β-monooxygenase
EC number1.14.15.4
CAS number9029-66-7
IntEnzIntEnz view
ExPASyNiceZyme view
MetaCycmetabolic pathway
PDB structuresRCSB PDB PDBe PDBsum
Gene OntologyAmiGO / QuickGO

It generates cortisol from 11-deoxycortisol and corticosterone from 11-deoxycorticosterone. Note the extra "–OH" added at the 11 position (near the center, on ring "C"):

Mechanism of action[edit]

As a mitochondrial P450 system, P450c11 is dependent on two electron transfer proteins, adrenodoxin reductase and adrenodoxin that transfer 2 electrons from NADPH to the P450 for each monooxygenase reaction catalyzed by the enzyme. In most respects this process of electron transfer appears similar to that of P450scc system that catalyzes cholesterol side chain cleavage.[9] Similar to P450scc the process of electrons transfer is leaky leading to superoxide production. The rate of electron leakage during metabolism depends on the functional groups of the steroid substrate.[10]


The expression of the enzyme in adrenocortical cells is regulated by the trophic hormone corticotropin (ACTH).[11]

Clinical significance[edit]

A mutation is associated with congenital adrenal hyperplasia due to 11β-hydroxylase deficiency.

See also[edit]

Additional images[edit]

Steroidogenesis, showing steroid 11-beta-hydroxylase vertically at right.


  1. ^ a b c GRCh38: Ensembl release 89: ENSG00000160882 - Ensembl, May 2017
  2. ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000022589 - Ensembl, May 2017
  3. ^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. ^ Lifton RP, Dluhy RG, Powers M, Rich GM, Gutkin M, Fallo F, Gill JR Jr, Feld L, Ganguly A (Jun 1993). "Hereditary hypertension caused by chimaeric gene duplications and ectopic expression of aldosterone synthase". Nat Genet. 2 (1): 66–74. doi:10.1038/ng0992-66. PMID 1303253.
  6. ^ a b "Entrez Gene: CYP11B1 cytochrome P450, family 11, subfamily B, polypeptide 1".
  7. ^ Dörr HG, Kuhnle U, Holthausen H, Bidlingmaier F, Knorr D (November 1984). "Etomidate: a selective adrenocortical 11 beta-hydroxylase inhibitor". Klinische Wochenschrift. 62 (21): 1011–3. doi:10.1007/bf01711722. PMID 6096625.
  8. ^ Carol L. Lake (7 December 2004). Pediatric Cardiac Anesthesia. Lippincott Williams & Wilkins. p. 68. ISBN 978-0-7817-5175-9. Retrieved 30 April 2012.
  9. ^ Hanukoglu I, Privalle CT, Jefcoate CR (May 1981). "Mechanisms of ionic activation of adrenal mitochondrial cytochromes P-450scc and P-45011 beta" (PDF). J. Biol. Chem. 256 (9): 4329–35. PMID 6783659.
  10. ^ Rapoport R, Sklan D, Hanukoglu I (March 1995). "Electron leakage from the adrenal cortex mitochondrial P450scc and P450c11 systems: NADPH and steroid dependence". Arch. Biochem. Biophys. 317 (2): 412–6. doi:10.1006/abbi.1995.1182. PMID 7893157.
  11. ^ Hanukoglu I, Feuchtwanger R, Hanukoglu A (November 1990). "Mechanism of corticotropin and cAMP induction of mitochondrial cytochrome P450 system enzymes in adrenal cortex cells" (PDF). J. Biol. Chem. 265 (33): 20602–8. PMID 2173715.

Further reading[edit]

External links[edit]