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GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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SPSB2
splA/ryanodine receptor domain and SOCS box containing 2
Chromosome 12 · 12p13.31
NCBI Gene: 84727Ensembl: ENSG00000111671.11HGNC: HGNC:29522UniProt: Q99619
35PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingubiquitin-like ligase-substrate adaptor activityubiquitin-dependent protein catabolic processprotein ubiquitinationneurodegenerative diseasecolorectal cancerduodenal ulcerneoplasm
✦AI Summary

SPSB2 functions as a substrate recognition component of an ECS (Elongin BC-CUL2/5-SOCS-box protein) E3 ubiquitin ligase complex that mediates ubiquitination and proteasomal degradation of target proteins 1. Its primary role is negatively regulating nitric oxide (NO) production by mediating ubiquitination and degradation of inducible NOS2 (iNOS), thereby limiting cellular toxicity in activated macrophages 2. SPSB2 acts as a bridge linking iNOS with ECS complex components ELOC and CUL5 2. Beyond NO regulation, SPSB2 targets diverse substrates: it inhibits hepatitis C virus replication by degrading the viral NS5A protein 3, and clears iNOS protein aggregates formed after Hsp90 inhibition through ubiquitin-proteasome-mediated degradation 4. Clinically, SPSB2 expression correlates with poor prognosis in hepatocellular carcinoma patients and is associated with tumor proliferation, invasion, and metastasis 5. The SPRY domain of SPSB2 recognizes specific substrate motifs (exemplified by DINNN sequences in iNOS) 6, making SPSB2 a target for therapeutic inhibition through cyclic peptide antagonists designed to augment NO production for antimicrobial and anticancer applications 7. MDM2-mediated degradation of SPSB2 stabilizes iNOS and promotes M1 macrophage inflammatory responses 8.

Sources cited
1
SPSB2 is a substrate recognition component of ECS E3 ubiquitin ligase complex
PMID: 15601820
2
SPSB2 negatively regulates NO production by mediating NOS2 ubiquitination and degradation; acts as bridge between NOS2 and ECS complex components
PMID: 21199876
3
SPSB2 inhibits hepatitis C virus replication by targeting NS5A for ubiquitination and degradation
PMID: 31344133
4
SPSB2 is essential for ubiquitination and proteasomal clearance of iNOS aggregates after Hsp90 inhibition
PMID: 29367003
5
SPSB2 expression correlates with poor prognosis and promotes proliferation, invasion, and metastasis in hepatocellular carcinoma
PMID: 40149497
6
SPSB2 SPRY domain recognizes substrate motifs like DINNN with specificity differences compared to other SPSB proteins
PMID: 20561531
7
SPSB2-iNOS interaction inhibitors increase NO levels with potential antimicrobial and anticancer applications
PMID: 30226743
8
MDM2-mediated SPSB2 degradation stabilizes iNOS and promotes M1 macrophage inflammatory responses
PMID: 39366973
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.31Weak
colorectal cancerOpen Targets
0.21Weak
duodenal ulcerOpen Targets
0.09Suggestive
neoplasmOpen Targets
0.08Suggestive
ThrombocytopeniaOpen Targets
0.06Suggestive
autosomal dominant macrothrombocytopeniaOpen Targets
0.05Suggestive
macrothrombocytopenia, isolated, 2, autosomal dominantOpen Targets
0.05Suggestive
thrombocytopenia 4Open Targets
0.05Suggestive
liver cancerOpen Targets
0.04Suggestive
congenital amegakaryocytic thrombocytopenia 1Open Targets
0.04Suggestive
thrombocytopenia 7Open Targets
0.04Suggestive
Beta-thalassemia - X-linked thrombocytopeniaOpen Targets
0.04Suggestive
beta-thalassemia-X-linked thrombocytopenia syndromeOpen Targets
0.04Suggestive
platelet-type bleeding disorder 9Open Targets
0.04Suggestive
platelet-type bleeding disorder 15Open Targets
0.04Suggestive
X-linked sideroblastic anemia 1Open Targets
0.04Suggestive
autoimmune thrombocytopenic purpuraOpen Targets
0.04Suggestive
thrombocytopenia 2Open Targets
0.04Suggestive
monosomy 7 myelodysplasia and leukemia syndrome 1Open Targets
0.03Suggestive
bleeding disorder, platelet-type, 24Open Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
NOS2Protein interaction87%CISHProtein interaction78%CUL5Protein interaction78%PAWRProtein interaction76%ELOCProtein interaction73%RNF7Protein interaction72%
Tissue Expression6 tissues
Lung
100%
Bone Marrow
57%
Liver
53%
Ovary
40%
Heart
26%
Brain
21%
Gene Interaction Network
Click a node to explore
SPSB2NOS2CISHCUL5PAWRELOCRNF7
PROTEIN STRUCTURE
Preparing viewer…
PDB6JWM · 1.23 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.63LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.18 [0.86–1.63]
RankingsWhere SPSB2 stands among ~20K protein-coding genes
  • #11,074of 20,598
    Most Researched35
  • #15,801of 17,882
    Most Constrained (LOEUF)1.63
Genes detectedSPSB2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
MDM2 induces pro-inflammatory and glycolytic responses in M1 macrophages by integrating iNOS-nitric oxide and HIF-1α pathways in mice.
PMID: 39366973
Nat Commun · 2024
1.00
2
The Prognostic Value and Immunomodulatory Role of Spsb2, a Novel Immune Checkpoint Molecule, in Hepatocellular Carcinoma.
PMID: 40149497
Genes (Basel) · 2025
0.90
3
Subtle Structural Differences Affect the Inhibitory Potency of RGD-Containing Cyclic Peptide Inhibitors Targeting SPSB Proteins.
PMID: 38928469
Int J Mol Sci · 2024
0.80
4
SPSB2 inhibits hepatitis C virus replication by targeting NS5A for ubiquitination and degradation.
PMID: 31344133
PLoS One · 2019
0.70
5
Crystal structure of SPSB2 in complex with a rational designed RGD-containing cyclic peptide inhibitor of SPSB2-iNOS interaction.
PMID: 28549582
Biochem Biophys Res Commun · 2017
0.60