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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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UBA3
ubiquitin like modifier activating enzyme 3
Chromosome 3 · 3p14.1
NCBI Gene: 9039Ensembl: ENSG00000144744.18HGNC: HGNC:12470UniProt: F8W8D4
89PubMed Papers
20Diseases
1Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Hub Gene
CLINICAL
Clinical Trials
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
NEDD8 activating enzyme activityprotein neddylationnucleusprotein bindingdengue diseaseacute myeloid leukemiamyelodysplastic syndromeinfectious meningitis
✦AI Summary

UBA3 is the catalytic subunit of the E1 NEDD8-activating enzyme complex (with NAE1/APPBP1), which catalyzes the first committed step in neddylation 1. UBA3 adenylates NEDD8's C-terminal glycine using ATP and transfers the activated NEDD8 to the E2 enzyme UBE2M via a thioester intermediate 12. This post-translational modification regulates cullin-RING ubiquitin ligases, affecting numerous cellular processes including cell cycle progression and protein degradation. Clinically, UBA3 dysregulation is implicated in multiple malignancies. UBA3 is highly expressed in intrahepatic cholangiocarcinoma, where knockdown inhibits proliferation, invasion, and migration through MAPK signaling 3. In lung adenocarcinoma, elevated UBA3 promotes an immunosuppressive microenvironment via NF-κB pathway activation, recruiting immunosuppressive cells and facilitating tumor immune escape 4. UBA3 is also co-dependent with PIK3CA mutations in head and neck squamous cell carcinoma, suggesting neddylation as a therapeutic target 5. Additionally, UBA3 supports interferon-stimulated gene expression in antiviral immunity through interaction with the prefoldin chaperone complex 6. In adult T-cell leukemia, neddylation pathway inhibition via UBA3 suppression increases PD-L1 expression, enhancing immunotherapy efficacy 7. These findings establish UBA3 as a critical hub in cell proliferation, immunity, and cancer pathogenesis.

Sources cited
1
UBA3-APPBP1 heterodimeric complex catalyzes NEDD8 adenylation and transfer to E2 enzyme Ubc12
PMID: 16275315
2
Biochemical characterization of NEDD8 activation mechanism by AppBp1-Uba3, including thioester intermediate formation
PMID: 12740388
3
UBA3 highly expressed in intrahepatic cholangiocarcinoma; knockdown inhibits proliferation, invasion, migration via MAPK pathway
PMID: 38298057
4
UBA3 promotes immunosuppressive tumor microenvironment in lung adenocarcinoma via NF-κB signaling and cytokine secretion
PMID: 37656220
5
UBA3 identified as genetic dependency in PIK3CA-mutated head and neck cancer through CRISPR screening
PMID: 40154000
6
UBA3 assists in interferon-stimulated gene expression through prefoldin complex interaction in antiviral immunity
PMID: 40883306
7
Neddylation pathway including UBA3 negatively regulates PD-L1 expression in adult T-cell leukemia/lymphoma
PMID: 38142436
8
UBA3 and UBE2M levels regulate CUL3 neddylation and WNK4 degradation in kidney, affecting blood pressure
PMID: 39798916
Disease Associationsⓘ20
dengue diseaseOpen Targets
0.37Weak
acute myeloid leukemiaOpen Targets
0.31Weak
myelodysplastic syndromeOpen Targets
0.29Weak
infectious meningitisOpen Targets
0.27Weak
neurodegenerative diseaseOpen Targets
0.21Weak
neoplasmOpen Targets
0.08Suggestive
intrahepatic cholangiocarcinomaOpen Targets
0.08Suggestive
non-small cell lung carcinomaOpen Targets
0.07Suggestive
celiac diseaseOpen Targets
0.07Suggestive
insomniaOpen Targets
0.06Suggestive
ankylosing spondylitisOpen Targets
0.06Suggestive
atrial fibrillationOpen Targets
0.05Suggestive
parasitic infectionOpen Targets
0.05Suggestive
lung adenocarcinomaOpen Targets
0.05Suggestive
breast cancerOpen Targets
0.04Suggestive
ovarian dysfunctionOpen Targets
0.04Suggestive
cancerOpen Targets
0.04Suggestive
metastatic melanomaOpen Targets
0.04Suggestive
multiple myelomaOpen Targets
0.04Suggestive
diaphragm diseaseOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Drug Targets1
PEVONEDISTATPhase III
NEDD8 activating enzyme inhibitor
myelodysplastic syndrome
Related Genes
UCHL3Protein interaction100%ATG7Protein interaction100%RBX1Protein interaction100%UBE2D2Protein interaction100%CUL7Protein interaction100%CUL4AProtein interaction100%
Tissue Expression6 tissues
Heart
100%
Brain
46%
Bone Marrow
38%
Lung
34%
Ovary
30%
Liver
24%
Gene Interaction Network
Click a node to explore
UBA3UCHL3ATG7RBX1UBE2D2CUL7CUL4A
PROTEIN STRUCTURE
Preparing viewer…
PDB5JJM · 2.15 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.46Moderately Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.29 [0.19–0.46]
RankingsWhere UBA3 stands among ~20K protein-coding genes
  • #5,389of 20,598
    Most Researched89
  • #2,629of 17,882
    Most Constrained (LOEUF)0.46 · top quartile
Genes detectedUBA3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Expression, purification, and characterization of the E1 for human NEDD8, the heterodimeric APPBP1-UBA3 complex.
PMID: 16275315
Methods Enzymol · 2005
1.00
2
UBA3 promotes the occurrence and metastasis of intrahepatic cholangiocarcinoma through MAPK signaling pathway.
PMID: 38298057
Acta Biochim Biophys Sin (Shanghai) · 2024
0.90
3
Camk2n1 deficiency reduces the NaCl cotransporter activity through the CUL3/KLHL3/WNK4 complex in the kidney.
PMID: 39798916
Eur J Pharmacol · 2025
0.80
4
Neddylation as a target in PIK3CA-mutated head and neck cancer.
PMID: 40154000
Biochem Biophys Res Commun · 2025
0.70
5
Prefoldin complex promotes interferon-stimulated gene expression and is inhibited by rotavirus VP3.
PMID: 40883306
Nat Commun · 2025
0.60