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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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SIAH2
siah E3 ubiquitin protein ligase 2
Chromosome 3 · 3q25.1
NCBI Gene: 6478Ensembl: ENSG00000181788.5HGNC: HGNC:10858UniProt: O43255
133PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
canonical Wnt signaling pathwaynegative regulation of canonical Wnt signaling pathwayregulation of circadian rhythmnegative regulation of apoptotic processbreast carcinomabreast cancerestrogen-receptor positive breast cancerprostate carcinoma
✦AI Summary

SIAH2 is a RING E3 ubiquitin ligase that mediates ubiquitination and proteasomal degradation of diverse substrate proteins 1. The enzyme functions by accepting ubiquitin from E2 enzymes and transferring it to target substrates either through direct binding or as a component of larger E3 complexes 1. SIAH2 regulates multiple cellular processes including transcription, apoptosis, cell cycle control, and circadian rhythm by targeting substrates such as transcriptional regulators (PML, NCOR1), cell surface receptors (DCC), and circadian repressors (NR1D1, NR1D2) 12. A critical function involves hypoxia regulation, where SIAH2 degrades prolyl hydroxylases PHD1 and PHD3, allowing HIF-1α accumulation under hypoxic conditions 3. SIAH2 exhibits context-dependent roles in cancer, promoting tumorigenesis in prostate, lung, gastric, and liver cancers while suppressing tumor progression in hepatocellular carcinoma through WNK1 degradation 45. Additionally, SIAH2 regulates adipogenesis by targeting the antiadipogenic protein ZFP521 for degradation 6 and controls neuronal differentiation by antagonizing DCC receptor signaling in cerebellar development 7. These diverse functions underscore SIAH2's importance as a regulatory hub in cellular homeostasis and disease pathogenesis.

Sources cited
1
SIAH2 is an E3 ubiquitin ligase that mediates ubiquitination and proteasomal degradation of target proteins including transcriptional regulators and cell surface receptors
PMID: 11483518
2
SIAH2 regulates prolyl hydroxylases PHD1 and PHD3 under hypoxia to allow HIF-1α accumulation
PMID: 19372575
3
SIAH2 regulates circadian rhythm through ubiquitination and degradation of NR1D1 and NR1D2
PMID: 26392558
4
SIAH2 promotes tumorigenesis in prostate, lung, gastric, and liver cancers but can exhibit tumor suppressor function in different cancer contexts
PMID: 34687788
5
SIAH2 acts as a tumor suppressor in hepatocellular carcinoma by targeting WNK1 for degradation
PMID: 36780970
6
SIAH2 is expressed in adipocyte precursor cells and promotes adipogenesis by targeting ZFP521 for degradation
PMID: 33155406
7
SIAH2 antagonizes Pard3/JamC to modulate DCC signaling during cerebellar granule neuron germinal zone exit
PMID: 39774925
Disease Associationsⓘ20
breast carcinomaOpen Targets
0.48Moderate
breast cancerOpen Targets
0.43Moderate
estrogen-receptor positive breast cancerOpen Targets
0.41Moderate
prostate carcinomaOpen Targets
0.31Weak
smoking initiationOpen Targets
0.31Weak
Benign Thyroid Gland NeoplasmOpen Targets
0.24Weak
neoplasmOpen Targets
0.11Weak
posterior cortical atrophyOpen Targets
0.09Suggestive
cancerOpen Targets
0.09Suggestive
hepatocellular carcinomaOpen Targets
0.09Suggestive
lung cancerOpen Targets
0.08Suggestive
skeletal system diseaseOpen Targets
0.08Suggestive
cholangiocarcinomaOpen Targets
0.08Suggestive
HypoglycemiaOpen Targets
0.07Suggestive
Gorham-Stout diseaseOpen Targets
0.07Suggestive
chronic myelogenous leukemiaOpen Targets
0.06Suggestive
squamous cell carcinomaOpen Targets
0.05Suggestive
gastric cancerOpen Targets
0.05Suggestive
pulmonary arterial hypertensionOpen Targets
0.04Suggestive
infectionOpen Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
HIF1AProtein interaction100%SNCAProtein interaction100%EGLN3Protein interaction98%LATS2Protein interaction96%EGLN2Protein interaction95%EGLN1Protein interaction95%
Tissue Expression6 tissues
Liver
100%
Bone Marrow
56%
Ovary
46%
Brain
42%
Lung
35%
Heart
33%
Gene Interaction Network
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SIAH2HIF1ASNCAEGLN3LATS2EGLN2EGLN1
PROTEIN STRUCTURE
Preparing viewer…
PDB5H9M · 1.76 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.46Moderately Constrained
pLIⓘ
0.99Intolerant
Observed/Expected LoF0.20 [0.10–0.46]
RankingsWhere SIAH2 stands among ~20K protein-coding genes
  • #3,499of 20,598
    Most Researched133 · top quartile
  • #2,596of 17,882
    Most Constrained (LOEUF)0.46 · top quartile
Genes detectedSIAH2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
The role of Siah2 in tumorigenesis and cancer therapy.
PMID: 34687788
Gene · 2022
1.00
2
The ubiquitin ligase Siah2 and the hypoxia response.
PMID: 19372575
Mol Cancer Res · 2009
0.90
3
The Siah2-HIF-FoxA2 axis in prostate cancer – new markers and therapeutic opportunities.
PMID: 21037926
Oncotarget · 2010
0.80
4
SIAH2 is Expressed in Adipocyte Precursor Cells and Interacts with EBF1 and ZFP521 to Promote Adipogenesis.
PMID: 33155406
Obesity (Silver Spring) · 2021
0.70
5
The inducible E3 ubiquitin ligases SIAH1 and SIAH2 perform critical roles in breast and prostate cancers.
PMID: 26028498
Cytokine Growth Factor Rev · 2015
0.60