AKR1C2

Chr 10AR

aldo-keto reductase family 1 member C2

Also known as: AKR1C-pseudo, BABP, DD, DD-2, DD/BABP, DD2, DDH2, HAKRD

This gene encodes a member of the aldo/keto reductase superfamily, which consists of more than 40 known enzymes and proteins. These enzymes catalyze the conversion of aldehydes and ketones to their corresponding alcohols using NADH and/or NADPH as cofactors. The enzymes display overlapping but distinct substrate specificity. This enzyme binds bile acid with high affinity, and shows minimal 3-alpha-hydroxysteroid dehydrogenase activity. This gene shares high sequence identity with three other gene members and is clustered with those three genes at chromosome 10p15-p14. Three transcript variants encoding two different isoforms have been found for this gene. [provided by RefSeq, Dec 2011]

OMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

46XY sex reversal 8MIM #614279
AR
UniProt46,XY sex reversal 8
1
Active trials
41
Pubs (1 yr)
P/LP submissions
P/LP missense
1.46
LOEUF
Multiple*
Mechanism· predicted

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.46LOEUF
pLI 0.000
Z-score 0.14
OE 0.96 (0.651.46)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.87Z-score
OE missense 1.20 (1.061.36)
176 obs / 146.3 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.96 (0.651.46)
00.351.4
Missense OE?1.20 (1.061.36)
00.61.4
Synonymous OE?1.31
01.21.6
LoF obs/exp: 16 / 16.6Missense obs/exp: 176 / 146.3Syn Z: -1.87

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

AKR1C2 · protein map & ClinVar variants

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