HomeAboutRankingsData Sources
Β© 2026 GeneE
🧬
GeneE
25 sources retrieved Β· Most recent: April 2026 Β· Index updated 15 days ago
β“˜GeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
SRD5A2
steroid 5 alpha-reductase 2
Chromosome 2 Β· 2p23.1
NCBI Gene: 6716Ensembl: ENSG00000277893.3HGNC: HGNC:11285UniProt: P31213
272PubMed Papers
21Diseases
3Drugs
93Pathogenic Variants
RESEARCH IMPACT
Variant-Rich
CLINICAL
FDA Approved TargetOMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
male gonad development3-oxo-5-alpha-steroid 4-dehydrogenase (NADP+) activitytestosterone biosynthetic processprotein binding46,XY disorder of sex development due to 5-alpha-reductase 2 deficiencyandrogenetic alopeciabenign prostatic hyperplasiaprostate carcinoma
✦AI Summary

SRD5A2 encodes steroid 5-alpha-reductase type 2, a critical enzyme that converts testosterone into 5-alpha-dihydrotestosterone (DHT), a more potent androgen 1. This conversion is essential for male sexual differentiation and urethral development during fetal growth 2. The enzyme is particularly important in ventral urethral tissues where it shapes male genital anatomy 2. Clinically, SRD5A2 loss-of-function mutations cause pseudovaginal perineoscrotal hypospadias, a congenital condition where the urethral opening is abnormally positioned 2. Genetic polymorphisms in SRD5A2, particularly the V89L variant, increase hypospadias risk significantly (OR=2.46 in dominant model) 2. The C allele at this locus functions as a genetic risk factor for disease occurrence 2. Regarding cancer associations, multiple large meta-analyses found no significant association between SRD5A2 polymorphisms and prostate cancer risk 341, despite initial smaller studies suggesting potential links. Similarly, V89L and TA repeat polymorphisms showed no association with breast cancer susceptibility 5. However, SRD5A2 variants influence metabolite profiles and may contribute to adverse drug effects, highlighting broader metabolic roles 6.

Sources cited
1
SRD5A2 encodes enzyme converting testosterone to dihydrotestosterone; A49T variant shows no association with prostate cancer risk
PMID: 18469342
2
SRD5A2 expressed in ventral urethra during male genital development; V89L polymorphism increases hypospadias risk with C allele as risk factor
PMID: 28713005
3
Meta-analysis of 24 studies found V89L not associated with prostate cancer; A49T probably not associated with prostate cancer
PMID: 19914946
4
Meta-analysis excluding V89L as risk factor for prostate cancer; A49T and TA repeat polymorphisms may have modest effects but cannot exclude bias
PMID: 12869400
5
V89L and TA repeat polymorphisms of SRD5A2 show no significant associations with breast cancer risk
PMID: 26345832
6
SRD5A2 variants influence human plasma metabolome and may contribute to adverse drug effects
PMID: 36357675
Disease Associationsβ“˜21
46,XY disorder of sex development due to 5-alpha-reductase 2 deficiencyOpen Targets
0.84Strong
androgenetic alopeciaOpen Targets
0.70Strong
benign prostatic hyperplasiaOpen Targets
0.62Moderate
prostate carcinomaOpen Targets
0.60Moderate
alopeciaOpen Targets
0.55Moderate
prostate cancerOpen Targets
0.53Moderate
prostate neoplasmOpen Targets
0.47Moderate
hypotrichosisOpen Targets
0.46Moderate
MicropenisOpen Targets
0.42Moderate
neoplasmOpen Targets
0.41Moderate
prostate adenocarcinomaOpen Targets
0.35Weak
hypogonadismOpen Targets
0.34Weak
tricuspid valve diseaseOpen Targets
0.32Weak
goutOpen Targets
0.29Weak
skin agingOpen Targets
0.28Weak
disease of genitourinary systemOpen Targets
0.27Weak
autism spectrum disorderOpen Targets
0.26Weak
sialolithiasisOpen Targets
0.26Weak
male reproductive system diseaseOpen Targets
0.21Weak
Abruptio PlacentaeOpen Targets
0.19Weak
Pseudovaginal perineoscrotal hypospadiasUniProt
Pathogenic Variants93
NM_000348.4(SRD5A2):c.377A>G (p.Gln126Arg)Pathogenic
not provided|3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency
β˜…β˜…β˜†β˜†2026β†’ Residue 126
NM_000348.4(SRD5A2):c.692A>G (p.His231Arg)Pathogenic
3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency|not provided
β˜…β˜…β˜†β˜†2026β†’ Residue 231
NM_000348.4(SRD5A2):c.737G>A (p.Arg246Gln)Pathogenic
3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency|SRD5A2-related disorder
β˜…β˜…β˜†β˜†2026β†’ Residue 246
NM_000348.4(SRD5A2):c.211C>T (p.Gln71Ter)Pathogenic
3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency
β˜…β˜…β˜†β˜†2025β†’ Residue 71
NM_000348.4(SRD5A2):c.680G>A (p.Arg227Gln)Pathogenic
Micropenis|not provided|3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency|Autism spectrum disorder|SRD5A2-related disorder
β˜…β˜…β˜†β˜†2025β†’ Residue 227
NM_000348.4(SRD5A2):c.164T>C (p.Leu55Pro)Pathogenic
not provided|3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency
β˜…β˜…β˜†β˜†2025β†’ Residue 55
NM_000348.4(SRD5A2):c.679C>T (p.Arg227Ter)Pathogenic
3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency
β˜…β˜…β˜†β˜†2025β†’ Residue 227
NM_000348.4(SRD5A2):c.682G>A (p.Ala228Thr)Pathogenic
3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency|not provided|Differences in sex development
β˜…β˜…β˜†β˜†2025β†’ Residue 228
NM_000348.4(SRD5A2):c.358A>C (p.Thr120Pro)Pathogenic
3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency|Differences in sex development
β˜…β˜…β˜†β˜†2025β†’ Residue 120
NM_000348.4(SRD5A2):c.578A>G (p.Asn193Ser)Pathogenic
3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 193
NM_000348.4(SRD5A2):c.704A>T (p.Tyr235Phe)Pathogenic
3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency|See cases
β˜…β˜…β˜†β˜†2025β†’ Residue 235
NM_000348.4(SRD5A2):c.547G>A (p.Gly183Ser)Pathogenic
3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency|SRD5A2-related disorder|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 183
NM_000348.4(SRD5A2):c.620C>A (p.Ala207Asp)Pathogenic
3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency
β˜…β˜…β˜†β˜†2025β†’ Residue 207
NM_000348.4(SRD5A2):c.344G>A (p.Gly115Asp)Pathogenic
3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency
β˜…β˜…β˜†β˜†2025β†’ Residue 115
NM_000348.4(SRD5A2):c.589G>A (p.Glu197Lys)Pathogenic
3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency
β˜…β˜…β˜†β˜†2025β†’ Residue 197
NM_000348.4(SRD5A2):c.586G>A (p.Gly196Ser)Pathogenic
3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency|not provided|SRD5A2-related disorder
β˜…β˜…β˜†β˜†2025β†’ Residue 196
NM_000348.4(SRD5A2):c.271T>C (p.Tyr91His)Pathogenic
3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 91
NM_000348.4(SRD5A2):c.282-2A>GPathogenic
3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency|not provided
β˜…β˜…β˜†β˜†2024
NM_000348.4(SRD5A2):c.383A>G (p.Tyr128Cys)Pathogenic
3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency
β˜…β˜…β˜†β˜†2024β†’ Residue 128
NM_000348.4(SRD5A2):c.433C>T (p.Arg145Trp)Pathogenic
3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency|not provided
β˜…β˜…β˜†β˜†2024β†’ Residue 145
View on ClinVar β†—
Drug Targets3
ABIRATERONEApproved
Androgen Receptor antagonist
prostate cancer
DUTASTERIDEApproved
Steroid 5-alpha-reductase inhibitor
androgenetic alopecia
FINASTERIDEApproved
Steroid 5-alpha-reductase 2 inhibitor
androgenetic alopecia
Related Genes
AKR1C3Protein interaction98%CYP11B1Protein interaction96%CYP11B2Protein interaction96%CYP19A1Protein interaction96%HSD3B1Protein interaction96%HSD17B2Protein interaction96%
Tissue Expression6 tissues
Liver
100%
Lung
1%
Ovary
0%
Brain
0%
Bone Marrow
0%
Heart
0%
Gene Interaction Network
Click a node to explore
SRD5A2AKR1C3CYP11B1CYP11B2CYP19A1HSD3B1HSD17B2
PROTEIN STRUCTURE
Preparing viewer…
PDB7BW1 Β· 2.80 Γ… Β· X-ray
View on RCSB β†—
Constraintβ“˜
LOEUFβ“˜
1.72LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF1.23 [0.88–1.72]
RankingsWhere SRD5A2 stands among ~20K protein-coding genes
  • #1,356of 20,598
    Most Researched272 Β· top 10%
  • #457of 1,025
    FDA-Approved Drug Targets3
  • #822of 5,498
    Most Pathogenic Variants93 Β· top quartile
  • #16,184of 17,882
    Most Constrained (LOEUF)1.72
Genes detectedSRD5A2
Sources retrieved25 papers
Response timeβ€”
πŸ“„ Sources
25β–Ό
1
Rare and common genetic determinants of metabolic individuality and their effects on human health.
PMID: 36357675
Nat Med Β· 2022
1.00
2
Steroid 5-alpha-reductase type 2 (SRD5A2) gene V89L polymorphism and hypospadias risk: A meta-analysis.
PMID: 28713005
J Pediatr Urol Β· 2017
0.90
3
Enhanced prostatic Esr1
PMID: 38606616
J Pathol Β· 2024
0.84
4
Associations between the SRD5A2 gene V89L and TA repeat polymorphisms and breast cancer risk: a meta-analysis.
PMID: 26345832
Genet Mol Res Β· 2015
0.80
5
Whole-exome sequencing study of hypospadias.
PMID: 37168556
iScience Β· 2023
0.72