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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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GTF2A1L
general transcription factor IIA subunit 1 like
Chromosome 2 · 2p16.3
NCBI Gene: 11036Ensembl: ENSG00000242441.8HGNC: HGNC:30727UniProt: Q9UNN4
23PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transcription Factor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
cognitionprotein bindingnucleoplasmtranscription by RNA polymerase IILeydig cell hypoplasia, type 1peripheral precocious pubertyLeydig cell hypoplasiadisorder of sexual differentiation
✦AI Summary

GTF2A1L (general transcription factor IIA subunit 1 like) functions as a testis-specific transcription factor involved in male fertility and reproductive development. The protein binds DNA in conjunction with GTF2A2 and TBP (TATA-binding protein), enabling mRNA transcription by RNA polymerase II 1. GTF2A1L expression is regulated by tissue-specific DNA methylation patterns, with aberrant methylation of its promoter region associated with hypospermatogenesis in males with non-obstructive azoospermia 2. However, while altered GTF2A1L expression correlates with testicular dysfunction, it does not appear to affect fertilization rates in testicular sperm extraction procedures 2. Single nucleotide polymorphisms in GTF2A1L are associated with FSH levels in azoospermic patients, suggesting a role in hormonal regulation of spermatogenesis 1. Additionally, the gene has been implicated in neurodevelopmental disorders, with altered expression observed in neural progenitors from fragile X syndrome patients 3. GTF2A1L variants have also been identified in genome-wide association studies of age-related macular degeneration bilaterality and polycystic ovary syndrome, indicating broader roles in disease susceptibility beyond male fertility 45.

Sources cited
1
GTF2A1L functions as a testis-specific transcription factor and SNPs in this gene correlate with FSH levels in azoospermic patients
PMID: 25374392
2
GTF2A1L promoter methylation is associated with hypospermatogenesis but does not affect fertilization rates in TESE procedures
PMID: 23770943
3
GTF2A1L expression is altered in neural progenitors from fragile X syndrome patients
PMID: 36506088
4
GTF2A1L variants are associated with bilateral neovascular age-related macular degeneration
PMID: 28775256
5
GTF2A1L is implicated in polycystic ovary syndrome through GWAS studies
PMID: 26305227
Disease Associationsⓘ20
Leydig cell hypoplasia, type 1Open Targets
0.56Moderate
peripheral precocious pubertyOpen Targets
0.55Moderate
Leydig cell hypoplasiaOpen Targets
0.47Moderate
disorder of sexual differentiationOpen Targets
0.43Moderate
Genetic 46,XY disorder of sex developmentOpen Targets
0.43Moderate
Precocious puberty in malesOpen Targets
0.40Weak
pseudohermaphroditismOpen Targets
0.33Weak
neurodegenerative diseaseOpen Targets
0.23Weak
genetic disorderOpen Targets
0.19Weak
Hypergonadotropic hypogonadismOpen Targets
0.18Weak
Abnormality of the skeletal systemOpen Targets
0.16Weak
46,XY disorder of sex developmentOpen Targets
0.11Weak
Aplasia cutis congenita - intestinal lymphangiectasiaOpen Targets
0.03Suggestive
aplasia cutis congenita-intestinal lymphangiectasia syndromeOpen Targets
0.03Suggestive
cardiac transplantOpen Targets
0.03Suggestive
congenital enteropathy due to enteropeptidase deficiencyOpen Targets
0.03Suggestive
congenital lethal erythrodermaOpen Targets
0.03Suggestive
restless legs syndromeOpen Targets
0.03Suggestive
MenorrhagiaOpen Targets
0.03Suggestive
OligomenorrheaOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
GTF2A1Protein interaction100%GTF2BProtein interaction100%GTF2A2Protein interaction97%TBPL1Protein interaction97%TBPL2Protein interaction91%STON1Protein interaction64%
Tissue Expression6 tissues
Ovary
100%
Lung
57%
Heart
0%
Bone Marrow
0%
Liver
0%
Brain
0%
Gene Interaction Network
Click a node to explore
GTF2A1LGTF2A1GTF2BGTF2A2TBPL1TBPL2STON1
PROTEIN STRUCTURE
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AlphaFoldAI-predicted · UniProt Q9UNN4
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.22LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.94 [0.74–1.22]
RankingsWhere GTF2A1L stands among ~20K protein-coding genes
  • #13,429of 20,598
    Most Researched23
  • #12,845of 17,882
    Most Constrained (LOEUF)1.22
Genes detectedGTF2A1L
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Reduced
PMID: 36506088
Front Cell Dev Biol · 2022
1.00
2
A genome-wide association study identified a novel genetic loci STON1-GTF2A1L/LHCGR/FSHR for bilaterality of neovascular age-related macular degeneration.
PMID: 28775256
Sci Rep · 2017
0.90
3
Association of single nucleotide polymorphisms in the USF1, GTF2A1L and OR2W3 genes with non-obstructive azoospermia in the Chinese population.
PMID: 25374392
J Assist Reprod Genet · 2015
0.80
4
Aberrant methylation of the TDMR of the GTF2A1L promoter does not affect fertilisation rates via TESE in patients with hypospermatogenesis.
PMID: 23770943
Asian J Androl · 2013
0.70
5
How to make an egg: transcriptional regulation in oocytes.
PMID: 15733063
Differentiation · 2005
0.60