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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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UBXN6
UBX domain protein 6
Chromosome 19 · 19p13.3
NCBI Gene: 80700Ensembl: ENSG00000167671.13HGNC: HGNC:14928UniProt: Q9BZV1
78PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingATPase bindingmitophagylate endosome membraneAbnormality of the skeletal systemneurodegenerative diseasedevice complicationAlzheimer disease
✦AI Summary

UBXN6 (UBX domain protein 6) functions as a critical cofactor for VCP/p97, an AAA-ATPase that regulates protein quality control and organellar homeostasis. UBXN6 negatively regulates VCP ATPase activity and participates in multiple cellular degradation pathways, including endoplasmic reticulum-associated degradation (ERAD) and autophagy 1. It mediates CAV1 transport to lysosomes for degradation and coordinates VCP-dependent clearance of damaged lysosomes 23. In immune cells, UBXN6 serves as an autophagy activator that suppresses inflammatory responses, with upregulation negatively correlating with inflammatory gene profiles in sepsis patients 4. UBXN6 also positively regulates JAK-STAT signaling by stabilizing TYK2 and type I IFNR during viral infection, enhancing interferon-stimulated gene expression 5. In mitophagy, UBXN6 facilitates PRKN recruitment and mitochondrial quality control by promoting mitofusin removal 6. A SNP in UBXN6 (rs1127888) associates with HIV non-progression phenotypes, modulating CAV1 trafficking and viral replication capacity 7. Dysregulation of UBXN6 contributes to pathologies including neurodegenerative diseases and head and neck cancer, where it correlates with endoplasmic reticulum stress responses and tumor immune microenvironment composition 89.

Sources cited
1
UBXN6 negatively regulates VCP ATPase activity as a cofactor
PMID: 26475856
2
UBXN6 participates in CAV1 transport to lysosomes for degradation
PMID: 21822278
3
UBXN6 role in CAV1 lysosomal degradation pathway
PMID: 23335559
4
UBXN6 activates autophagy and suppresses inflammation in macrophages; upregulation in sepsis negatively correlates with inflammatory genes
PMID: 39438692
5
UBXN6 positively regulates JAK-STAT1/2 signaling by stabilizing TYK2 and type I IFNR during viral infection
PMID: 34021047
6
UBXN6 facilitates PRKN recruitment and MFN2 removal during mitophagy
PMID: 33966597
7
UBXN6 SNP rs1127888 associates with HIV non-progression and modulates CAV1 trafficking and viral replication
PMID: 31158522
8
UBXN6 associates with poly-GA aggregates in C9orf72-related neurodegeneration and is involved in sequestration of molecular chaperones
PMID: 35164882
9
UBXN6 is part of an endoplasmic reticulum stress-related signature in head and neck squamous carcinoma with prognostic implications
PMID: 39043683
Disease Associationsⓘ20
Abnormality of the skeletal systemOpen Targets
0.39Weak
neurodegenerative diseaseOpen Targets
0.37Weak
device complicationOpen Targets
0.22Weak
Alzheimer diseaseOpen Targets
0.11Weak
multiple sclerosisOpen Targets
0.05Suggestive
response to polyunsaturated fatty acid supplementationOpen Targets
0.04Suggestive
SepsisOpen Targets
0.03Suggestive
LeishmaniasisOpen Targets
0.01Suggestive
infectionOpen Targets
0.01Suggestive
common wartOpen Targets
0.01Suggestive
bacterial diseaseOpen Targets
0.01Suggestive
Huntington diseaseOpen Targets
0.01Suggestive
ataxia telangiectasiaOpen Targets
0.01Suggestive
autoimmune diseaseOpen Targets
0.00Suggestive
ovarian endometrioid carcinomaOpen Targets
0.00Suggestive
visceral LeishmaniasisOpen Targets
0.00Suggestive
viral diseaseOpen Targets
0.00Suggestive
type 1 diabetes mellitusOpen Targets
0.00Suggestive
amyotrophic lateral sclerosisOpen Targets
0.00Suggestive
early-onset generalized limb-onset dystoniaOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
UBXN10Protein interaction100%UBXN2AProtein interaction95%SVIPProtein interaction93%VCPKMTProtein interaction85%UBXN8Protein interaction84%NSFL1CProtein interaction84%
Tissue Expression6 tissues
Liver
100%
Ovary
98%
Lung
76%
Heart
75%
Brain
73%
Bone Marrow
42%
Gene Interaction Network
Click a node to explore
UBXN6UBXN10UBXN2ASVIPVCPKMTUBXN8NSFL1C
PROTEIN STRUCTURE
Preparing viewer…
PDB8FCN · 2.95 Å · EM
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.17LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.91 [0.72–1.17]
RankingsWhere UBXN6 stands among ~20K protein-coding genes
  • #6,119of 20,598
    Most Researched78
  • #12,217of 17,882
    Most Constrained (LOEUF)1.17
Genes detectedUBXN6
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Ubiquitin regulatory X (UBX) domain-containing protein 6 is essential for autophagy induction and inflammation control in macrophages.
PMID: 39438692
Cell Mol Immunol · 2024
1.00
2
Linear ubiquitination at damaged lysosomes induces local NFKB activation and controls cell survival.
PMID: 39744815
Autophagy · 2025
0.90
3
UBX Domain Protein 6 Positively Regulates JAK-STAT1/2 Signaling.
PMID: 34021047
J Immunol · 2021
0.80
4
VCP/p97 cofactor UBXN1/SAKS1 regulates mitophagy by modulating MFN2 removal from mitochondria.
PMID: 33966597
Autophagy · 2022
0.70
5
VCP maintains lysosomal homeostasis and TFEB activity in differentiated skeletal muscle.
PMID: 30654731
Autophagy · 2019
0.60