CYP11B1

Chr 8ARAD

cytochrome P450 family 11 subfamily B member 1

Also known as: CPN1, CYP11B, FHI, P450C11

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]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Adrenal hyperplasia, congenital, due to 11-beta-hydroxylase deficiencyMIM #202010
AR
Aldosteronism, glucocorticoid-remediableMIM #103900
AD
UniProtHyperaldosteronism, familial, 1
5
Active trials
94
Pubs (1 yr)
P/LP submissions
P/LP missense
0.95
LOEUF
Multiple*
Mechanism· predicted
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.95LOEUF
pLI 0.000
Z-score 1.72
OE 0.60 (0.390.95)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
-1.56Z-score
OE missense 1.26 (1.151.37)
367 obs / 291.9 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.60 (0.390.95)
00.351.4
Missense OE?1.26 (1.151.37)
00.61.4
Synonymous OE?1.47
01.21.6
LoF obs/exp: 13 / 21.6Missense obs/exp: 367 / 291.9Syn Z: -4.03

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CYP11B1 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.