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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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PIAS2
protein inhibitor of activated STAT 2
Chromosome 18 · 18q21.1
NCBI Gene: 9063Ensembl: ENSG00000078043.17HGNC: HGNC:17311UniProt: O75928
146PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Highly ConstrainedTranscription Factor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
PML bodynucleusprotein sumoylationprotein bindingneurodegenerative diseasebrain aneurysmaneurysmalcohol drinking
✦AI Summary

PIAS2 (protein inhibitor of activated STAT 2) is an E3 SUMO ligase that functions as a transcriptional coregulator across multiple cellular pathways. It catalyzes protein sumoylation by stabilizing interactions between UBE2I and substrates, with effects varying by isoform and biological context 1. PIAS2 plays critical roles in regulating JAK-STAT signaling, p53 pathway, steroid hormone signaling, and DNA damage responses by modifying target proteins including hSSB1, MDM2, and ELK1 2. Dysregulated PIAS2 expression associates with multiple diseases. Elevated neuronal PIAS2 impairs interferon-β signaling and mitochondrial homeostasis, contributing to sporadic Parkinson disease dementia through blocked mitophagy and oxidative stress accumulation 3. PIAS2 expression correlates with multiple sclerosis severity and disability progression 4. In anaplastic thyroid carcinomas and other aggressive cancers, PIAS2β is dosage-sensitive and required for mitotic spindle assembly; selective depletion triggers mitotic catastrophe while sparing normal cells 5. PIAS2 also promotes melanoma through TRAF2-mediated EPHA5 sumoylation, enhancing receptor stability and signaling 6. Additionally, PIAS2α mediates huANP32A/B sumoylation facilitating avian influenza virus adaptation 7, and PIAS2 dysregulation involves diabetic nephropathy pathogenesis 8. These findings establish PIAS2 as a multifunctional regulator with therapeutic targeting potential in neurodegenerative, autoimmune, and malignant diseases.

Sources cited
1
PIAS2 elevation in neurons blocks IFN-β signaling, impairs mitochondrial homeostasis and mitophagy, and causes motor/cognitive impairments and dopaminergic neuron loss contributing to Parkinson disease dementia
PMID: 34234281
2
PIAS2 gene expression is elevated in multiple sclerosis patients and significantly correlates with disease severity
PMID: 31084243
3
PIAS2α specifically fine-tunes hSSB1 sumoylation at K79 and K94, which stabilizes hSSB1 and enhances NBS1 recruitment to DNA damage sites
PMID: 32576812
4
PIAS2 is upregulated in podocytes of diabetic nephropathy patients and mice, involved in the pathogenesis of diabetic nephropathy
PMID: 36251411
5
PIAS2 (ARIP3) is a member of the PIAS protein family that interacts with androgen receptor zinc-finger region and modulates AR-dependent transactivation
PMID: 10961928
6
PIAS2α SUMOylates human ANP32A/B proteins, facilitating NS2 SUMO-interacting motif binding and promoting avian influenza virus polymerase activity
PMID: 39737943
7
PIAS2β is required for mitotic spindle assembly and is dosage-sensitive in anaplastic thyroid carcinomas; depletion triggers mitotic catastrophe while sparing normal thyroid cells
PMID: 38744818
8
PIAS2 functions as SUMO E3 ligase to sumoylate EPHA5, suppressing ubiquitination and degradation, thereby promoting IL-17-induced melanoma proliferation and invasion
PMID: 37481078
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.37Weak
aneurysmOpen Targets
0.31Weak
brain aneurysmOpen Targets
0.31Weak
alcohol drinkingOpen Targets
0.27Weak
preeclampsiaOpen Targets
0.24Weak
ovarian dysfunctionOpen Targets
0.22Weak
chronic obstructive pulmonary diseaseOpen Targets
0.08Suggestive
azoospermiaOpen Targets
0.08Suggestive
Alzheimer diseaseOpen Targets
0.07Suggestive
partial chromosome Y deletionOpen Targets
0.06Suggestive
Testicular regression syndromeOpen Targets
0.06Suggestive
myelodysplastic syndromeOpen Targets
0.06Suggestive
spermatogenic failure 25Open Targets
0.05Suggestive
isochromosomy YpOpen Targets
0.05Suggestive
spermatogenic failure 71Open Targets
0.05Suggestive
spermatogenic failure 57Open Targets
0.05Suggestive
spermatogenic failure 50Open Targets
0.05Suggestive
spermatogenic failure 63Open Targets
0.05Suggestive
Isolated follicle stimulating hormone deficiencyOpen Targets
0.05Suggestive
46,XY complete gonadal dysgenesisOpen Targets
0.05Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
SAE1Protein interaction100%STAT1Protein interaction100%STAT5BProtein interaction100%STAT5AProtein interaction100%TP53Protein interaction99%MYCProtein interaction99%
Tissue Expression6 tissues
Heart
100%
Brain
78%
Bone Marrow
66%
Ovary
65%
Liver
56%
Lung
56%
Gene Interaction Network
Click a node to explore
PIAS2SAE1STAT1STAT5BSTAT5ATP53MYC
PROTEIN STRUCTURE
Preparing viewer…
PDB4FO9 · 2.39 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.32Highly Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.18 [0.10–0.32]
RankingsWhere PIAS2 stands among ~20K protein-coding genes
  • #3,114of 20,598
    Most Researched146 · top quartile
  • #1,259of 17,882
    Most Constrained (LOEUF)0.32 · top 10%
Genes detectedPIAS2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
PIAS2-mediated blockade of IFN-β signaling: a basis for sporadic Parkinson disease dementia.
PMID: 34234281
Mol Psychiatry · 2021
1.00
2
The investigation of relevancy between
PMID: 31084243
J Immunoassay Immunochem · 2019
0.90
3
SUMOylation stabilizes hSSB1 and enhances the recruitment of NBS1 to DNA damage sites.
PMID: 32576812
Signal Transduct Target Ther · 2020
0.80
4
Integrative transcriptome analysis reveals TEKT2 and PIAS2 involvement in diabetic nephropathy.
PMID: 36251411
FASEB J · 2022
0.70
5
Androgen-receptor-interacting nuclear proteins.
PMID: 10961928
Biochem Soc Trans · 2000
0.60