DHRS11

Chr 17

dehydrogenase/reductase 11

Also known as: ARPG836, SDR24C1, spDHRS11

The protein encoded by DHRS11 is a steroid dehydrogenase that catalyzes the conversion of steroid hormones, including the reduction of androgen precursors to potent androgens like 11-ketotestosterone and the interconversion of estrone/estradiol. Mutations cause autosomal recessive 46,XY differences of sex development with undervirilization due to impaired androgen biosynthesis. This gene is highly constrained against loss-of-function variants (pLI near 1.0), indicating that complete loss of protein function is likely deleterious.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 1.33
Clinical SummaryDHRS11
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.33LOEUF
pLI 0.000
Z-score 0.71
OE 0.78 (0.481.33)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.53Z-score
OE missense 0.87 (0.751.02)
122 obs / 139.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.78 (0.481.33)
00.351.4
Missense OE0.87 (0.751.02)
00.61.4
Synonymous OE0.73
01.21.6
LoF obs/exp: 10 / 12.8Missense obs/exp: 122 / 139.5Syn Z: 1.61
DN
0.7327th %ile
GOF
0.5856th %ile
LOF
0.2872th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DHRS11 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →