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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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UBXN7
UBX domain protein 7
Chromosome 3 · 3q29
NCBI Gene: 26043Ensembl: ENSG00000163960.14HGNC: HGNC:29119UniProt: O94888
69PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Highly ConstrainedHub Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
nucleoplasmVCP-NPL4-UFD1 AAA ATPase complexubiquitin bindingRNA polymerase II-specific DNA-binding transcription factor bindingneurodegenerative diseaseAbnormal nasolacrimal system morphologysmoking initiationhypertrophic cardiomyopathy
✦AI Summary

UBXN7 (UBX domain protein 7) is a multifunctional adapter protein that coordinates ubiquitin-mediated protein degradation and cellular stress responses. Its primary function involves linking cullin-RING ligases (CRLs) to the p97/VCP segregase complex to regulate substrate turnover 1. UBXN7 operates through multiple mechanisms: it directly binds neddylated cullins via its UIM motif, independently of ubiquitin binding 2, and reduces the ubiquitin threshold required for p97-UFD1-NPL4 unfoldase activity, facilitating efficient substrate unfolding 3. The protein serves as a scaffold for both CRL3KEAP1 and CRL2VHL complexes, reciprocally regulating NRF2 and HIF-1α transcription factors in response to oxidative stress and hypoxia 1. UBXN7 is essential for DNA replication termination, participating in replisome disassembly through CUL2LRR1-driven MCM7 ubiquitylation 4. Disease relevance includes its role in viral replication, where it stabilizes SARS-CoV-2 N protein by inhibiting K48-linked ubiquitination 5, and in hepatitis B infection, where HBx protein induces UBXN7 degradation to activate NF-κB signaling 6. Additionally, UBXN7 shows potential as a diagnostic biomarker in pulmonary hypertension 7.

Sources cited
1
UBXN7 serves as a scaffold for CRL3KEAP1 and CRL2VHL complexes and reciprocally regulates NRF2 and HIF-1α
PMID: 33444648
2
UBXN7 reduces the ubiquitin threshold of p97-UFD1-NPL4 unfoldase and is essential for CMG disassembly
PMID: 35920641
3
UBXN7 promotes SARS-CoV-2 replication by inhibiting K48-linked ubiquitination of viral N protein
PMID: 41086194
4
UBXN7 is required for replisome disassembly in human cells through CUL2LRR1-driven MCM7 ubiquitylation
PMID: 39055910
5
UBXN7 directly interacts with neddylated cullins via its UIM motif and causes HIF1α accumulation
PMID: 22537386
6
UBXN7 is upregulated in pulmonary hypertension and shows potential as a diagnostic biomarker
PMID: 36740764
7
HBx protein induces UBXN7 degradation to activate NF-κB signaling and promote HBV replication
PMID: 36096451
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.54Moderate
Abnormal nasolacrimal system morphologyOpen Targets
0.27Weak
smoking initiationOpen Targets
0.19Weak
hypertrophic cardiomyopathyOpen Targets
0.12Weak
tooth diseaseOpen Targets
0.10Weak
NephropathyOpen Targets
0.08Suggestive
nephritisOpen Targets
0.07Suggestive
breast cancerOpen Targets
0.02Suggestive
hepatocellular carcinomaOpen Targets
0.01Suggestive
diabetic nephropathyOpen Targets
0.01Suggestive
nonpapillary renal cell carcinomaOpen Targets
0.01Suggestive
colorectal cancerOpen Targets
0.01Suggestive
Pick diseaseOpen Targets
0.01Suggestive
juvenile dermatomyositisOpen Targets
0.01Suggestive
Alzheimer diseaseOpen Targets
0.00Suggestive
COVID-19–associated multisystem inflammatory syndrome in adultsOpen Targets
0.00Suggestive
hepatitis B virus infectionOpen Targets
0.00Suggestive
lumbar disc degenerationOpen Targets
0.00Suggestive
neoplasmOpen Targets
0.00Suggestive
oral squamous cell carcinomaOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
KBTBD13Shared pathway100%LRRC75AShared pathway100%KLHL30Shared pathway100%KLHL38Shared pathway100%KLHL10Shared pathway100%KLHL35Shared pathway100%
Tissue Expression6 tissues
Bone Marrow
100%
Brain
80%
Ovary
38%
Lung
32%
Heart
31%
Liver
17%
Gene Interaction Network
Click a node to explore
UBXN7KBTBD13LRRC75AKLHL30KLHL38KLHL10KLHL35
PROTEIN STRUCTURE
Preparing viewer…
PDB5X3P · 2.00 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.28Highly Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.16 [0.09–0.28]
RankingsWhere UBXN7 stands among ~20K protein-coding genes
  • #6,866of 20,598
    Most Researched69
  • #960of 17,882
    Most Constrained (LOEUF)0.28 · top 10%
Genes detectedUBXN7
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
UBXN7 cofactor of CRL3
PMID: 33444648
Biochim Biophys Acta Mol Cell Res · 2021
1.00
2
Multiple UBX proteins reduce the ubiquitin threshold of the mammalian p97-UFD1-NPL4 unfoldase.
PMID: 35920641
Elife · 2022
0.90
3
UBXN7 facilitates SARS-CoV-2 replication via inhibiting the K48-linked ubiquitination of viral N protein.
PMID: 41086194
PLoS Pathog · 2025
0.80
4
Characterizing replisome disassembly in human cells.
PMID: 39055910
iScience · 2024
0.70
5
UBXN7 docks on neddylated cullin complexes using its UIM motif and causes HIF1α accumulation.
PMID: 22537386
BMC Biol · 2012
0.60