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GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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DMRTB1
DMRT like family B with proline rich C-terminal 1
Chromosome 1 · 1p32.3
NCBI Gene: 63948Ensembl: ENSG00000143006.7HGNC: HGNC:13913UniProt: I6L9A0
20PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transcription Factor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingDNA-binding transcription factor activity, RNA polymerase II-specificRNA polymerase II cis-regulatory region sequence-specific DNA bindingnucleusadolescent idiopathic scoliosisobesityDecreased total leukocyte countdeficiency anemia
✦AI Summary

DMRTB1 (DMRT6) is a transcription factor belonging to the conserved doublesex and mab-3-related (DMRT) gene family 1, characterized by a DNA-binding domain and proline-rich C-terminus. It functions as an RNA polymerase II-specific transcription factor with chr1-regulatory activity. Functionally, DMRTB1 plays a pivotal role in coordinating the mitosis-to-meiosis transition in germ cells 2. In human testis, DMRTB1 is specifically expressed in spermatogonia (particularly A-pale and B subtypes) and primary spermatocytes at early meiotic stages 2. In female germ cell development, DMRTB1 expression is regulated downstream of DMRTA2 during oogonial differentiation prior to meiotic entry 3. Clinically, altered DMRTB1 expression patterns correlate with spermatogenic arrest at the spermatogonial level, while its absence in germ cell neoplasia in situ and seminoma cells suggests a protective role against malignant transformation 2. At the genomic level, disruption of a topologically associated domain boundary near DMRTB1 reduces testicular gene expression and spermatogenesis efficiency by 15-18% 4. A familial 1p32 microdeletion encompassing DMRTB1 associates with learning difficulties, developmental delay, and ambiguous genitalia, indicating importance in sexual development 5. These findings establish DMRTB1 as essential for normal germ cell development and sexual differentiation.

Sources cited
1
DMRTB1 is expressed in spermatogonia and primary spermatocytes; altered expression correlates with spermatogenic arrest and absent in germ cell tumors
PMID: 30920770
2
DMRTB1 is a member of the conserved DMRT gene family containing a DNA-binding domain, evolutionarily related across species
PMID: 15478606
3
DMRTB1 (DMRT6) expression is regulated downstream of DMRTA2 in human female fetal germ cells during oogonial differentiation and meiotic transition
PMID: 25082981
4
Disruption of TAD boundary near DMRTB1 causes altered testis gene expression and 15-18% reduction in spermatogenesis efficiency
PMID: 39903672
5
DMRTB1 is located in 1p32 deletion region associated with learning difficulties, developmental delay, and ambiguous genitalia in familial cases
PMID: 29240611
Disease Associationsⓘ20
adolescent idiopathic scoliosisOpen Targets
0.26Weak
obesityOpen Targets
0.18Weak
Decreased total leukocyte countOpen Targets
0.16Weak
deficiency anemiaOpen Targets
0.16Weak
response to statinOpen Targets
0.15Weak
DNA methylationOpen Targets
0.10Weak
azoospermiaOpen Targets
0.07Suggestive
Testicular regression syndromeOpen Targets
0.06Suggestive
46,XX testicular disorder of sex developmentOpen Targets
0.06Suggestive
46,XY complete gonadal dysgenesisOpen Targets
0.06Suggestive
rectosigmoid junction neoplasmOpen Targets
0.06Suggestive
46,XY disorder of sex development due to 5-alpha-reductase 2 deficiencyOpen Targets
0.06Suggestive
partial chromosome Y deletionOpen Targets
0.06Suggestive
spermatogenic failure 57Open Targets
0.06Suggestive
spermatogenic failure 50Open Targets
0.05Suggestive
46,XX ovotesticular disorder of sex developmentOpen Targets
0.05Suggestive
persistent Mullerian duct syndromeOpen Targets
0.05Suggestive
Persistent Müllerian duct syndromeOpen Targets
0.05Suggestive
spermatogenic failure 25Open Targets
0.05Suggestive
spermatogenic failure 71Open Targets
0.05Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
DMRT2Shared pathway100%DMRTA1Shared pathway100%DMRTA2Shared pathway100%CDKL2Shared pathway50%DMRT3Shared pathway50%TSPY1Shared pathway50%
Tissue Expression6 tissues
Bone Marrow
100%
Liver
0%
Brain
0%
Heart
0%
Ovary
0%
Lung
0%
Gene Interaction Network
Click a node to explore
DMRTB1DMRT2DMRTA1DMRTA2CDKL2DMRT3TSPY1
PROTEIN STRUCTURE
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AlphaFoldAI-predicted · UniProt Q96MA1
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.72LoF Tolerant
pLIⓘ
0.24Tolerant
Observed/Expected LoF0.42 [0.26–0.72]
RankingsWhere DMRTB1 stands among ~20K protein-coding genes
  • #14,144of 20,598
    Most Researched20
  • #5,510of 17,882
    Most Constrained (LOEUF)0.72
Genes detectedDMRTB1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Immunolocalization of DMRTB1 in human testis with normal and impaired spermatogenesis.
PMID: 30920770
Andrology · 2019
1.00
2
Deletion of an evolutionarily conserved TAD boundary impacts spermatogenesis in mice†.
PMID: 39903672
Biol Reprod · 2025
0.90
3
[Cloning of four members of giant panda Dmrt genes].
PMID: 15478606
Yi Chuan Xue Bao · 2004
0.80
4
Association of host genome with intestinal microbial composition in a large healthy cohort.
PMID: 27694960
Nat Genet · 2016
0.70
5
Deletion of an evolutionarily conserved TAD boundary compromises spermatogenesis in mice.
PMID: 39026739
bioRxiv · 2024
0.60