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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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URI1
URI1 prefoldin like chaperone
Chromosome 19 · 19q12
NCBI Gene: 8725Ensembl: ENSG00000105176.19HGNC: HGNC:13236UniProt: O94763
96PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
OncogeneTranscription Factor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
nucleoplasmcytoplasmtranscription corepressor activityprotein bindingneurodegenerative diseaselung diseasemetabolic diseaseskull disorder
✦AI Summary

URI1 (unconventional prefoldin RPB5 interactor 1) is a prefoldin-like chaperone and scaffolding protein that regulates critical cellular processes including protein complex assembly, metabolic homeostasis, and tissue regeneration. URI1 functions as a core component of the PAQosome complex, which assembles macromolecular complexes and interacts with molecular chaperones 1. At the molecular level, URI1 binds and regulates glutamine synthase in hepatocytes, controlling glutamate levels to fine-tune liver regeneration through effects on macrophage metabolic reprogramming and YAP1-dependent hepatocyte proliferation 2. URI1 also modulates β-catenin signaling in intestinal stem cells, with reduced URI1 levels promoting c-MYC expression and tissue regeneration following ionizing radiation 3. Clinically, URI1 dysregulation associates with multiple diseases. URI1 is overexpressed in hepatocellular carcinoma, where HBx and c-MYC cooperatively induce its expression 4. In rheumatoid arthritis, lncRNA-mediated URI1 signaling promotes fibroblast-like synoviocyte proliferation and inflammation, which therapeutic agents can suppress 5. URI1 also participates in acute respiratory distress syndrome pathogenesis, with URI1 upregulation via CBX4 promoting mitophagy and reducing inflammation 6. Additionally, PAQosome components including URI1 regulate hepatic fibrosis through PI3K/AKT signaling 7, suggesting therapeutic potential in liver disease.

Sources cited
1
URI1 colocalizes with and activates glutamine synthase in pericentral hepatocytes, regulating glutamate levels to control liver regeneration
PMID: 40140584
2
URI1 is a core component of the PAQosome complex essential for assembly and stabilization of macromolecular complexes
PMID: 30484152
3
URI1 inhibits β-catenin signaling in intestinal label-retaining cells, regulating their proliferation and radiosensitivity after ionizing radiation
PMID: 31147493
4
URI1 is overexpressed in HBV-related hepatocellular carcinoma through c-MYC and HBx-mediated promoter activation
PMID: 31739577
5
lncRNA RP11-83J16.1-mediated URI1 signaling promotes rheumatoid arthritis fibroblast proliferation and inflammation; triptolide suppresses this pathway
PMID: 34358861
6
CBX4-mediated URI1 transcription promotes mitophagy and reduces inflammation and oxidative stress in acute respiratory distress syndrome
PMID: 40040620
7
PAQosome components including URI1 regulate hepatic fibrosis through PI3K/AKT signaling
PMID: 38016755
8
URI1 functions as a scaffold protein assembling prefoldin family members and binding RNA polymerase II subunit RPB5
PMID: 30484156
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.53Moderate
lung diseaseOpen Targets
0.32Weak
metabolic diseaseOpen Targets
0.32Weak
skull disorderOpen Targets
0.30Weak
Meniere diseaseOpen Targets
0.30Weak
diabetes mellitusOpen Targets
0.28Weak
Abnormality of the skeletal systemOpen Targets
0.15Weak
cervical carcinomaOpen Targets
0.12Weak
infectious arthritisOpen Targets
0.11Weak
hepatocellular carcinomaOpen Targets
0.11Weak
osteonecrosisOpen Targets
0.10Weak
poisoningOpen Targets
0.10Weak
neoplasmOpen Targets
0.10Suggestive
colorectal carcinomaOpen Targets
0.09Suggestive
urinary bladder cancerOpen Targets
0.09Suggestive
coronary artery calcificationOpen Targets
0.09Suggestive
breast benign neoplasmOpen Targets
0.09Suggestive
infectious diseaseOpen Targets
0.08Suggestive
Abruptio PlacentaeOpen Targets
0.08Suggestive
Miyoshi myopathyOpen Targets
0.08Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PIH1D1Protein interaction100%RUVBL2Protein interaction100%PFDN6Protein interaction100%HSP90AB1Protein interaction99%PDRG1Protein interaction99%PFDN2Protein interaction98%
Tissue Expression6 tissues
Brain
100%
Heart
54%
Bone Marrow
49%
Ovary
48%
Liver
32%
Lung
31%
Gene Interaction Network
Click a node to explore
URI1PIH1D1RUVBL2PFDN6HSP90AB1PDRG1PFDN2
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt O94763
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.84LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.63 [0.48–0.84]
RankingsWhere URI1 stands among ~20K protein-coding genes
  • #5,010of 20,598
    Most Researched96 · top quartile
  • #7,238of 17,882
    Most Constrained (LOEUF)0.84
Genes detectedURI1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Macrophages harness hepatocyte glutamate to boost liver regeneration.
PMID: 40140584
Nature · 2025
1.00
2
The Multiple Functions of the PAQosome: An R2TP- and URI1 Prefoldin-Based Chaperone Complex.
PMID: 30484152
Adv Exp Med Biol · 2018
0.90
3
The Yeast Prefoldin Bud27.
PMID: 30484156
Adv Exp Med Biol · 2018
0.80
4
Protocatechuic Acid Alleviates Inflammation and Oxidative Stress in Acute Respiratory Distress Syndrome by Promoting Unconventional Prefoldin RPB5 Interactor 1-Mediated Mitophagy.
PMID: 40040620
Chem Biol Drug Des · 2025
0.70
5
URI is required to maintain intestinal architecture during ionizing radiation.
PMID: 31147493
Science · 2019
0.60