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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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UPK3B
uroplakin 3B
Chromosome 7 · 7q11.23
NCBI Gene: 105375355Ensembl: ENSG00000243566.8HGNC: HGNC:21444UniProt: A0A0J9YVS6
22PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingmembraneplasma membraneneoplasmmetabolic syndromePituitary Gland Adenomalung cancer
✦AI Summary

UPK3B (uroplakin 3B) is a structural protein primarily functioning as a component of the asymmetric unit membrane (AUM) in terminally differentiated urothelial cells 1. It plays critical roles in AUM-cytoskeleton interactions and contributes to urothelial glycocalyx formation, which prevents bacterial adherence to the bladder epithelium 2. Beyond its canonical urological role, UPK3B serves as a selective marker for mesothelial cells and is expressed in retinal pigment epithelial (RPE) cells during development 34. In RPE differentiation, UPK3B expression is regulated downstream of MYRF transcription factor and is essential for maintaining cell structure and survival during retinal development 5. Clinically, UPK3B immunohistochemistry is diagnostically valuable, showing strong positivity in mesotheliomas (82.1% of epithelioid cases) while being largely absent in other malignancies, making it useful for distinguishing mesotheliomas from other thoracic tumors 1. Emerging evidence suggests UPK3B may function as an immunological biomarker; high expression in pancreatic adenocarcinoma correlates with reduced MHC expression, low immunogenicity, and poor response to immune checkpoint inhibitor therapy 6. Additionally, UPK3B is downregulated during gonadal development as coelomic epithelial cells differentiate into supporting gonadal cells 7, indicating tissue-specific developmental regulation.

Sources cited
1
UPK3B is involved in stabilizing urothelial cells; expressed primarily in mesothelial cells, urothelial umbrella cells, and amnion cells; useful diagnostic marker for mesothelioma
PMID: 36292206
2
UPK3B ortholog (upk3b) identified in zebrafish genome; expressed in urinary bladder contributing to urothelial function and glycocalyx formation
PMID: 37293698
3
UPK3B identified as marker of primary mesothelial cells; detected in retinal pigment epithelial cells and urothelial cells
PMID: 21984916
4
UPK3B is a structural target gene downregulated in MYRF knockout RPE; MYRF regulates UPK3B expression for RPE maturation and cell structure
PMID: 40233131
5
Upk3b is downregulated in MYRF knockout retinal pigment epithelium; essential for RPE maturation and cell structure
PMID: 38746430
6
UPK3B highly expressed in pancreatic adenocarcinoma correlates with low MHC expression, low immunogenicity, immunosuppressive cell infiltration, and poor ICI therapy response
PMID: 40223017
7
UPK3B downregulated at day 6 of gonadal development by FOXL2; marks transition from coelomic epithelium during sex determination
PMID: 40102860
Disease Associationsⓘ20
neoplasmOpen Targets
0.09Suggestive
metabolic syndromeOpen Targets
0.07Suggestive
Pituitary Gland AdenomaOpen Targets
0.07Suggestive
lung cancerOpen Targets
0.06Suggestive
experimental autoimmune encephalomyelitisOpen Targets
0.06Suggestive
severe malarial anemiaOpen Targets
0.06Suggestive
diffuse large B-cell lymphomaOpen Targets
0.05Suggestive
COVID-19Open Targets
0.05Suggestive
Alzheimer diseaseOpen Targets
0.05Suggestive
nasopharyngeal carcinomaOpen Targets
0.04Suggestive
non-small cell lung carcinomaOpen Targets
0.04Suggestive
systemic sclerodermaOpen Targets
0.04Suggestive
proliferative diabetic retinopathyOpen Targets
0.04Suggestive
esophageal adenocarcinomaOpen Targets
0.03Suggestive
ovarian carcinomaOpen Targets
0.03Suggestive
esophageal squamous cell carcinomaOpen Targets
0.03Suggestive
sarcoidosisOpen Targets
0.03Suggestive
infectionOpen Targets
0.03Suggestive
small cell lung carcinomaOpen Targets
0.03Suggestive
cancerOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
UPK1BProtein interaction94%UPK2Protein interaction94%UPK1AProtein interaction94%LRRN4Protein interaction74%ERMNCo-mentioned in literature20%
Tissue Expression6 tissues
Lung
100%
Ovary
8%
Bone Marrow
4%
Liver
4%
Heart
3%
Brain
0%
Gene Interaction Network
Click a node to explore
UPK3BUPK1BUPK2UPK1ALRRN4ERMN
PROTEIN STRUCTURE
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AlphaFoldAI-predicted · UniProt Q9BT76
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.24LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.85 [0.60–1.24]
RankingsWhere UPK3B stands among ~20K protein-coding genes
  • #13,790of 20,598
    Most Researched22
  • #13,089of 17,882
    Most Constrained (LOEUF)1.24
Genes detectedUPK3B
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Comprehensive analysis of UPK3B as a marker for prognosis and immunity in pancreatic adenocarcinoma.
PMID: 40223017
Sci Rep · 2025
1.00
2
FOXL2 drives the differentiation of supporting gonadal cells in early ovarian development.
PMID: 40102860
Reprod Biol Endocrinol · 2025
0.90
3
Myelin regulatory factor (MYRF) is a critical early regulator of retinal pigment epithelial development.
PMID: 40233131
PLoS Genet · 2025
0.80
4
LRRN4 and UPK3B are markers of primary mesothelial cells.
PMID: 21984916
PLoS One · 2011
0.70
5
Analysis of More than 16,000 Human Tumor and Normal Tissues Identifies Uroplakin 3B as a Useful Diagnostic Marker for Mesothelioma and Normal Mesothelial Cells.
PMID: 36292206
Diagnostics (Basel) · 2022
0.60