Gene Symbol: Upk3a
Description: uroplakin 3A
Alias: 1110017C07Rik, UP3a, UPIII, Upk3, uroplakin-3a, proplakin IIIa, uroplakin 3, uroplakin III
Species: mouse
Products:     Upk3a

Top Publications

  1. Wu X, Lin J, Walz T, Haner M, Yu J, Aebi U, et al. Mammalian uroplakins. A group of highly conserved urothelial differentiation-related membrane proteins. J Biol Chem. 1994;269:13716-24 pubmed
  2. Oostergetel G, Keegstra W, Brisson A. Structure of the major membrane protein complex from urinary bladder epithelial cells by cryo-electron crystallography. J Mol Biol. 2001;314:245-52 pubmed
    ..The total thickness of the complex is about 13.2 nm. A model of the urothelial plaque reveals that contacts between the hexamers are much less extended than within the hexamers. ..
  3. Hu P, Meyers S, Liang F, Deng F, Kachar B, Zeidel M, et al. Role of membrane proteins in permeability barrier function: uroplakin ablation elevates urothelial permeability. Am J Physiol Renal Physiol. 2002;283:F1200-7 pubmed
    ..To study this problem, we have ablated the mouse gene encoding uroplakin III (UPIII), one of the major protein subunits in urothelial apical membranes, and measured the permeabilities of ..
  4. Nie X, Sun J, Gordon R, Cai C, Xu P. SIX1 acts synergistically with TBX18 in mediating ureteral smooth muscle formation. Development. 2010;137:755-65 pubmed publisher
    ..These findings uncover an essential role for Six1 in establishing a functionally normal ureter and provide new insights into the molecular basis of urinary tract malformations in BOR patients. ..
  5. Yu Z, Mannik J, Soto A, Lin K, Andersen B. The epidermal differentiation-associated Grainyhead gene Get1/Grhl3 also regulates urothelial differentiation. EMBO J. 2009;28:1890-903 pubmed publisher
    ..These results show a crucial role for Get1 in urothelial differentiation and barrier formation. ..
  6. Kong X, Deng F, Hu P, Liang F, Zhou G, Auerbach A, et al. Roles of uroplakins in plaque formation, umbrella cell enlargement, and urinary tract diseases. J Cell Biol. 2004;167:1195-204 pubmed
    ..To study uroplakin function, we ablated the mouse UPII gene. A comparison of the phenotypes of UPII- and UPIII-deficient mice yielded new insights into the mechanism of plaque formation and some fundamental features of ..
  7. Hu P, Deng F, Liang F, Hu C, Auerbach A, Shapiro E, et al. Ablation of uroplakin III gene results in small urothelial plaques, urothelial leakage, and vesicoureteral reflux. J Cell Biol. 2000;151:961-72 pubmed
    ..We show that the ablation of the mouse uroplakin III (UPIII) gene leads to overexpression, defective glycosylation, and abnormal targeting of uroplakin Ib, the ..
  8. Airik R, Bussen M, Singh M, Petry M, Kispert A. Tbx18 regulates the development of the ureteral mesenchyme. J Clin Invest. 2006;116:663-74 pubmed
    ..Our analysis also showed that the ureteral mesenchyme derives from a distinct cell population that is separated early in kidney development from that of other mesenchymal cells of the renal system. ..
  9. Cheng W, Jacobs W, Zhang J, Moro A, Park J, Kushida M, et al. DeltaNp63 plays an anti-apoptotic role in ventral bladder development. Development. 2006;133:4783-92 pubmed
    ..We conclude that DeltaNp63 plays a crucial anti-apoptotic role in normal bladder development...

More Information


  1. Pignon J, Grisanzio C, Geng Y, Song J, Shivdasani R, Signoretti S. p63-expressing cells are the stem cells of developing prostate, bladder, and colorectal epithelia. Proc Natl Acad Sci U S A. 2013;110:8105-10 pubmed publisher
    ..Because p63 is a master regulator of stratified epithelial development, this finding provides a unique developmental insight into the cell of origin of squamous cell metaplasia and squamous cell carcinoma of the colon. ..
  2. Guha A, Vasconcelos M, Cai Y, Yoneda M, Hinds A, Qian J, et al. Neuroepithelial body microenvironment is a niche for a distinct subset of Clara-like precursors in the developing airways. Proc Natl Acad Sci U S A. 2012;109:12592-7 pubmed publisher
    ..precursors juxtaposed to presumptive neuroepithelial bodies (NEBs), distinguished by their strong Scgb3a2 and uroplakin 3a (Upk3a) signals and reduced Ccsp (Scgb1a1) expression...
  3. Zhou G, Liang F, Romih R, Wang Z, Liao Y, Ghiso J, et al. MAL facilitates the incorporation of exocytic uroplakin-delivering vesicles into the apical membrane of urothelial umbrella cells. Mol Biol Cell. 2012;23:1354-66 pubmed publisher
  4. Tai G, Ranjzad P, Marriage F, Rehman S, Denley H, Dixon J, et al. Cytokeratin 15 marks basal epithelia in developing ureters and is upregulated in a subset of urothelial cell carcinomas. PLoS ONE. 2013;8:e81167 pubmed publisher
    ..Superficial epithelial cells, including those positive for the differentiation marker uroplakin III, were CK15-...
  5. Vieira N, Deng F, Liang F, Liao Y, Chang J, Zhou G, et al. SNX31: a novel sorting nexin associated with the uroplakin-degrading multivesicular bodies in terminally differentiated urothelial cells. PLoS ONE. 2014;9:e99644 pubmed publisher
  6. Morimoto M, Nishinakamura R, Saga Y, Kopan R. Different assemblies of Notch receptors coordinate the distribution of the major bronchial Clara, ciliated and neuroendocrine cells. Development. 2012;139:4365-73 pubmed publisher
    ..These results suggest that two different assemblies of Notch receptors coordinate the numbers and distribution of the major epithelial cell types in the conducting airway during lung organogenesis. ..
  7. Zocher F, Zeidel M, Missner A, Sun T, Zhou G, Liao Y, et al. Uroplakins do not restrict CO2 transport through urothelium. J Biol Chem. 2012;287:11011-7 pubmed publisher
    ..The observed dependence of P(CO2) on CA activity suggests that the tightness of biological membranes to CO(2) may uniquely be regulated via CA expression. ..
  8. Cain J, Islam E, Haxho F, Chen L, Bridgewater D, Nieuwenhuis E, et al. GLI3 repressor controls nephron number via regulation of Wnt11 and Ret in ureteric tip cells. PLoS ONE. 2009;4:e7313 pubmed publisher
    ..Thus, GLI3 repressor controls nephron number by regulating ureteric tip cell expression of Wnt11 and Ret. ..
  9. Lee A, Polgar N, Napoli J, Lui V, Tamashiro K, Fujimoto B, et al. Fibroproliferative response to urothelial failure obliterates the ureter lumen in a mouse model of prenatal congenital obstructive nephropathy. Sci Rep. 2016;6:31137 pubmed publisher
    ..Our findings support the model that a defective urothelial barrier allows urine to induce a fibrotic wound healing mechanism, which may contribute to human prenatal UPJ obstructions. ..
  10. Mitchell E, Taylor D, Woods K, Davis M, Nelson A, Teasdale R, et al. Differential gene expression in the developing mouse ureter. Gene Expr Patterns. 2006;6:519-38 pubmed
    ..This study marks the first known report defining global gene expression of the developing mouse ureter and will provide insight into the molecular mechanisms underlying kidney and lower urinary tract malformations. ..
  11. Georgas K, Armstrong J, Keast J, Larkins C, McHugh K, Southard Smith E, et al. An illustrated anatomical ontology of the developing mouse lower urogenital tract. Development. 2015;142:1893-908 pubmed publisher
    ..The revised ontology will be an important tool for researchers studying urogenital development/malformation in mouse models and will improve our capacity to appropriately interpret these with respect to the human situation. ..
  12. Fogelgren B, Polgar N, Lui V, Lee A, Tamashiro K, Napoli J, et al. Urothelial Defects from Targeted Inactivation of Exocyst Sec10 in Mice Cause Ureteropelvic Junction Obstructions. PLoS ONE. 2015;10:e0129346 pubmed publisher
  13. Rudat C, Grieskamp T, Röhr C, Airik R, Wrede C, Hegermann J, et al. Upk3b is dispensable for development and integrity of urothelium and mesothelium. PLoS ONE. 2014;9:e112112 pubmed publisher
    ..Upk3b is coexpressed with the closely related Upk3a gene in the urothelium but not in the mesothelium, leaving the possibility of a functional redundancy between the ..
  14. Guha A, Deshpande A, Jain A, Sebastiani P, Cardoso W. Uroplakin 3a+ Cells Are a Distinctive Population of Epithelial Progenitors that Contribute to Airway Maintenance and Post-injury Repair. Cell Rep. 2017;19:246-254 pubmed publisher
    ..A recent screen for CC markers identified rare Uroplakin3a (Upk3a)-expressing cells (U-CCs) with a v-CC-like distribution...
  15. Stuart H, Roberts N, Hilton E, McKenzie E, Daly S, Hadfield K, et al. Urinary tract effects of HPSE2 mutations. J Am Soc Nephrol. 2015;26:797-804 pubmed publisher
    ..In conclusion, heparanase 2 is an autonomic neural protein implicated in bladder emptying, but HPSE2 variants are uncommon in urinary diseases resembling UFS. ..
  16. Weiss R, Guo S, Shan A, Shi H, Romano R, Sinha S, et al. Brg1 determines urothelial cell fate during ureter development. J Am Soc Nephrol. 2013;24:618-26 pubmed publisher
  17. Hicks A, Campeau L, Burmeister D, Bishop C, Andersson K. Lack of nicotinamide mononucleotide adenylyltransferase 2 (Nmnat2): consequences for mouse bladder development and function. Neurourol Urodyn. 2013;32:1130-6 pubmed publisher
    ..Moderate loss of Nmnat2 has no effect on bladder development, survival, and has only modest effects on bladder function later in life. ..
  18. Haraguchi R, Matsumaru D, Nakagata N, Miyagawa S, Suzuki K, Kitazawa S, et al. The hedgehog signal induced modulation of bone morphogenetic protein signaling: an essential signaling relay for urinary tract morphogenesis. PLoS ONE. 2012;7:e42245 pubmed publisher
    ..These results suggested that Hh-responsive mesenchymal Bmp signaling maintains the population of peri-cloacal mesenchyme cells, which is essential for the development of the ureter and the upper urinary tract. ..
  19. Zupancic D, Zakrajšek M, Zhou G, Romih R. Expression and localization of four uroplakins in urothelial preneoplastic lesions. Histochem Cell Biol. 2011;136:491-500 pubmed publisher
    ..Our study demonstrates that despite a lower level of expression, UPIa, UPIb, UPII and UPIIIa maintain their plaque association in urothelial preneoplastic lesions...
  20. Cain J, Islam E, Haxho F, Blake J, Rosenblum N. GLI3 repressor controls functional development of the mouse ureter. J Clin Invest. 2011;121:1199-206 pubmed publisher
    ..Together, these data demonstrate that Hh signaling controls Kit and Hcn3 expression and ureter peristalsis. ..
  21. Ryan A, Womack J, Yu J, Lin J, Wu X, Sun T, et al. Chromosomal localization of uroplakin genes of cattle and mice. Mamm Genome. 1993;4:656-61 pubmed
    ..Two bovine genomic DNA sequences reactive with a uroplakin III cDNA probe were identified and mapped to BTA 6 (UPK3A) and 5 (UPK3B)...
  22. Izgi K, Altuntas C, Bicer F, Ozer A, Sakalar C, Li X, et al. Uroplakin peptide-specific autoimmunity initiates interstitial cystitis/painful bladder syndrome in mice. PLoS ONE. 2013;8:e72067 pubmed publisher
    ..In this study, we show that the bladder specific uroplakin 3A-derived immunogenic peptide UPK3A 65-84, which contains the binding motif for IA(d) MHC class II molecules ..
  23. Mahoney Z, Sammut B, Xavier R, Cunningham J, Go G, Brim K, et al. Discs-large homolog 1 regulates smooth muscle orientation in the mouse ureter. Proc Natl Acad Sci U S A. 2006;103:19872-7 pubmed
    ..Our results suggest that (i) besides its well documented role in regulating epithelial polarity, Dlgh1 also regulates smooth muscle orientation, and (ii) human DLG1 mutations may contribute to hereditary forms of hydronephrosis. ..