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26 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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PIAS1
protein inhibitor of activated STAT 1
Chromosome 15 · 15q23
NCBI Gene: 8554Ensembl: ENSG00000033800.15HGNC: HGNC:2752UniProt: O75925
269PubMed Papers
20Diseases
0Drugs
1Pathogenic Variants
FUNCTIONAL ROLE
Highly ConstrainedTranscription Factor
RESEARCH IMPACT
Trending
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
positive regulation of transcription by RNA polymerase IItranscription regulator inhibitor activityprotein sumoylationnucleusAbnormality of the skeletal systemdiabetes mellitustype 2 diabetes mellitusuterine fibroid
✦AI Summary

PIAS1 (Protein Inhibitor of Activated STAT1) is a SUMO E3 ligase that functions as a critical regulator of protein SUMOylation and transcriptional control. Its primary role involves inhibiting STAT1 phosphorylation through enhanced SUMOylation, thereby modulating immune signaling and ferroptosis responses 1. PIAS1 restricts Epstein-Barr virus lytic replication by inhibiting transcription factors required for viral gene expression, though viral caspases can antagonize this restriction 2. In cancer contexts, PIAS1 functions as a coregulator of androgen receptor signaling in breast and prostate cancer, influencing cell survival and proliferation pathways 3. Importantly, circular RNA-derived PIAS1 variants promote hepatocellular carcinoma and melanoma progression by inhibiting ferroptosis through distinct mechanisms: HCC via the miR-455-3p/NUPR1/FTH1 axis 4, and melanoma by blocking immunogenic ferroptosis via STAT1 SUMOylation modulation 1. Therapeutically, targeting circPIAS1 combined with immunotherapy enhances treatment efficacy 1. Beyond cancer, PIAS1-mediated SUMOylation regulates ROCK2 activation in allergic asthma, promoting goblet cell metaplasia 5, and participates in DNA damage repair through the CK2/PIAS1/SPOP axis, affecting PARP inhibitor sensitivity in prostate cancer 6. PIAS1 also modulates SUMOylation-dependent mitophagy in cardiovascular disease 7.

Sources cited
1
Its primary role involves inhibiting STAT1 phosphorylation through enhanced SUMOylation, thereby modulating immune signaling and ferroptosis responses .
PMID: 39334380
2
PIAS1 restricts Epstein-Barr virus lytic replication by inhibiting transcription factors required for viral gene expression, though viral caspases can antagonize this restriction .
PMID: 29262325
3
In cancer contexts, PIAS1 functions as a coregulator of androgen receptor signaling in breast and prostate cancer, influencing cell survival and proliferation pathways .
PMID: 26219822
4
Importantly, circular RNA-derived PIAS1 variants promote hepatocellular carcinoma and melanoma progression by inhibiting ferroptosis through distinct mechanisms: HCC via the miR-455-3p/NUPR1/FTH1 axis , and melanoma by blocking immunogenic ferroptosis via STAT1 SUMOylation modulation .
PMID: 38802795
5
Beyond cancer, PIAS1-mediated SUMOylation regulates ROCK2 activation in allergic asthma, promoting goblet cell metaplasia , and participates in DNA damage repair through the CK2/PIAS1/SPOP axis, affecting PARP inhibitor sensitivity in prostate cancer .
PMID: 37393345
6
Beyond cancer, PIAS1-mediated SUMOylation regulates ROCK2 activation in allergic asthma, promoting goblet cell metaplasia , and participates in DNA damage repair through the CK2/PIAS1/SPOP axis, affecting PARP inhibitor sensitivity in prostate cancer .
PMID: 40260915
7
PIAS1 also modulates SUMOylation-dependent mitophagy in cardiovascular disease .
PMID: 36163375
Disease Associationsⓘ20
Abnormality of the skeletal systemOpen Targets
0.47Moderate
diabetes mellitusOpen Targets
0.33Weak
type 2 diabetes mellitusOpen Targets
0.33Weak
uterine fibroidOpen Targets
0.28Weak
osteoarthritis, kneeOpen Targets
0.27Weak
nephronophthisisOpen Targets
0.26Weak
Abruptio PlacentaeOpen Targets
0.25Weak
alcohol drinkingOpen Targets
0.22Weak
osteoarthritisOpen Targets
0.22Weak
osteoarthritis, hipOpen Targets
0.22Weak
central nervous system cancerOpen Targets
0.21Weak
liver diseaseOpen Targets
0.20Weak
AnisometropiaOpen Targets
0.20Weak
brain cancerOpen Targets
0.19Weak
ovarian carcinomaOpen Targets
0.15Weak
coronary artery bypassOpen Targets
0.11Weak
breast cancerOpen Targets
0.10Weak
neoplasmOpen Targets
0.10Weak
positive regulation of ovulationOpen Targets
0.10Suggestive
posterior cortical atrophyOpen Targets
0.09Suggestive
Pathogenic Variants1
NM_016166.3(PIAS1):c.317C>T (p.Ser106Leu)Likely pathogenic
Nephronophthisis
☆☆☆☆2015→ Residue 106
View on ClinVar ↗
Related Genes
SATB1Protein interaction100%TP53BP1Protein interaction100%MBD1Protein interaction100%STAT5BProtein interaction100%TP53Protein interaction100%STAT5AProtein interaction100%
Tissue Expression6 tissues
Bone Marrow
100%
Brain
99%
Lung
76%
Heart
73%
Ovary
66%
Liver
32%
Gene Interaction Network
Click a node to explore
PIAS1SATB1TP53BP1MBD1STAT5BTP53STAT5A
PROTEIN STRUCTURE
Preparing viewer…
PDB1V66 · NMR
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.14Highly Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.03 [0.01–0.14]
RankingsWhere PIAS1 stands among ~20K protein-coding genes
  • #1,376of 20,598
    Most Researched269 · top 10%
  • #4,678of 5,498
    Most Pathogenic Variants1
  • #158of 17,882
    Most Constrained (LOEUF)0.14 · top 1%
Genes detectedPIAS1
Sources retrieved26 papers
Response time—
📄 Sources
26▼
1
CircPIAS1 promotes hepatocellular carcinoma progression by inhibiting ferroptosis via the miR-455-3p/NUPR1/FTH1 axis.
PMID: 38802795
Mol Cancer · 2024
1.00
2
Pleiotropic effects of methionine adenosyltransferases deregulation as determinants of liver cancer progression and prognosis.
PMID: 23665184
J Hepatol · 2013
0.90
3
Circular RNA-encoded oncogenic PIAS1 variant blocks immunogenic ferroptosis by modulating the balance between SUMOylation and phosphorylation of STAT1.
PMID: 39334380
Mol Cancer · 2024
0.80
4
GATA2 promotes castration-resistant prostate cancer development by suppressing IFN-β axis-mediated antitumor immunity.
PMID: 39068217
Oncogene · 2024
0.76
5
SUMOylation targeting mitophagy in cardiovascular diseases.
PMID: 36163375
J Mol Med (Berl) · 2022
0.70