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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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SH3PXD2A
SH3 and PX domains 2A
Chromosome 10 · 10q24.33
NCBI Gene: 9644Ensembl: ENSG00000107957.17HGNC: HGNC:23664UniProt: B3KPL1
84PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingsuperoxide metabolic processosteoclast fusionpodosomeatrial fibrillationstrokeIschemic strokeatrial flutter
✦AI Summary

SH3PXD2A encodes Tks5, a scaffold protein that plays critical roles in cellular invasion and extracellular matrix remodeling through invadosome and podosome formation 1. The protein contains SH3 and PX domains and acts as an organizer for NADPH oxidase-dependent reactive oxygen species generation 1. The gene produces multiple isoforms, including Tks5β which lacks the PX domain and has distinct regulatory properties 1. In development, SH3PXD2A is essential for mammalian embryogenesis, as homozygous disruption results in neonatal death and cleft palate formation 1. The gene has significant disease relevance across multiple conditions. Genetic variants in SH3PXD2A are associated with stroke risk, particularly lacunar stroke, where it contributes to vascular extracellular matrix disruption 23. The gene is also implicated in intracerebral hemorrhage susceptibility 4 and cerebral white matter hyperintensities through DNA methylation mechanisms 5. In oncology, SH3PXD2A-HTRA1 fusion genes occur in 12.7% of intracranial schwannomas, particularly trigeminal schwannomas, and are associated with more aggressive tumor behavior and higher recurrence rates 6. Additionally, mutations in SH3PXD2A contribute to rosacea pathogenesis through neurogenic inflammation pathways involving vasoactive neuropeptide production 7. These findings establish SH3PXD2A as a key regulator of cellular invasion, vascular integrity, and tumor progression.

Sources cited
1
SH3PXD2A encodes Tks5 scaffold protein essential for invadosome formation, has multiple isoforms including Tks5β, and is required for mammalian development
PMID: 25259869
2
SH3PXD2A variants are associated with lacunar stroke through vascular extracellular matrix disruption
PMID: 33773637
3
SH3PXD2A is identified as a putative causal gene for stroke risk in cross-ancestry GWAS
PMID: 36180795
4
SH3PXD2A is associated with intracerebral hemorrhage susceptibility
PMID: 39298701
5
SH3PXD2A is implicated in cerebral white matter hyperintensities through DNA methylation mechanisms
PMID: 35943854
6
SH3PXD2A-HTRA1 fusion occurs in 12.7% of intracranial schwannomas and is associated with aggressive tumor behavior
PMID: 40971076
7
SH3PXD2A mutations contribute to rosacea through neurogenic inflammation and vasoactive neuropeptide production
PMID: 37402769
Disease Associationsⓘ20
atrial fibrillationOpen Targets
0.52Moderate
strokeOpen Targets
0.49Moderate
Ischemic strokeOpen Targets
0.48Moderate
atrial flutterOpen Targets
0.45Moderate
hypertensionOpen Targets
0.45Moderate
Uterine leiomyomaOpen Targets
0.44Moderate
uterine fibroidOpen Targets
0.44Moderate
cardiac arrhythmiaOpen Targets
0.44Moderate
diverticular diseaseOpen Targets
0.44Moderate
atrioventricular blockOpen Targets
0.44Moderate
heart conduction diseaseOpen Targets
0.42Moderate
asthmaOpen Targets
0.41Moderate
MenorrhagiaOpen Targets
0.38Weak
heart failureOpen Targets
0.38Weak
heart diseaseOpen Targets
0.38Weak
First degree atrioventricular blockOpen Targets
0.38Weak
Second degree atrioventricular blockOpen Targets
0.38Weak
retinal vascular disorderOpen Targets
0.38Weak
potassium deficiency diseaseOpen Targets
0.37Weak
chronic obstructive pulmonary diseaseOpen Targets
0.35Weak
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
CDC42Protein interaction100%NCK1Protein interaction100%GRB2Protein interaction100%CFL1Protein interaction99%CFL2Protein interaction99%CTTNProtein interaction99%
Tissue Expression6 tissues
Ovary
100%
Heart
67%
Brain
62%
Liver
49%
Lung
48%
Bone Marrow
11%
Gene Interaction Network
Click a node to explore
SH3PXD2ACDC42NCK1GRB2CFL1CFL2CTTN
PROTEIN STRUCTURE
Preparing viewer…
PDB2DNU · NMR
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.38Moderately Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.27 [0.20–0.38]
RankingsWhere SH3PXD2A stands among ~20K protein-coding genes
  • #5,697of 20,598
    Most Researched84
  • #1,786of 17,882
    Most Constrained (LOEUF)0.38 · top 10%
Genes detectedSH3PXD2A
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Stroke genetics informs drug discovery and risk prediction across ancestries.
PMID: 36180795
Nature · 2022
1.00
2
Genetic basis of lacunar stroke: a pooled analysis of individual patient data and genome-wide association studies.
PMID: 33773637
Lancet Neurol · 2021
0.90
3
Genetic and clinical characteristics of cranial nerve schwannoma harboring SH3PXD2A-HTRA1 fusion gene.
PMID: 40971076
Acta Neuropathol · 2025
0.80
4
Whole genome sequencing identifies genetic variants associated with neurogenic inflammation in rosacea.
PMID: 37402769
Nat Commun · 2023
0.70
5
Genetic disruption of the sh3pxd2a gene reveals an essential role in mouse development and the existence of a novel isoform of tks5.
PMID: 25259869
PLoS One · 2014
0.60