Gene Symbol: pkd2
Description: polycystic kidney disease 2
Alias: polycystin-2, TRPP2, cup, curly up, polycystic kidney disease 2 protein homolog, transient receptor potential cation channel subfamily P member 2
Species: zebrafish
Products:     pkd2

Top Publications

  1. Paavola J, Schliffke S, Rossetti S, Kuo I, Yuan S, Sun Z, et al. Polycystin-2 mutations lead to impaired calcium cycling in the heart and predispose to dilated cardiomyopathy. J Mol Cell Cardiol. 2013;58:199-208 pubmed publisher
    Mutations in PKD1 and PKD2, the genes encoding the proteins polycystin-1 (PC1) and polycystin-2 (PC2), cause autosomal dominant polycystic kidney disease (ADPKD)...
  2. Sun Z, Amsterdam A, Pazour G, Cole D, Miller M, Hopkins N. A genetic screen in zebrafish identifies cilia genes as a principal cause of cystic kidney. Development. 2004;131:4085-93 pubmed
    ..Two of these genes, vhnf1 (tcf2) and pkd2, are already associated with human cystic kidney diseases...
  3. Giamarchi A, Feng S, Rodat Despoix L, Xu Y, Bubenshchikova E, Newby L, et al. A polycystin-2 (TRPP2) dimerization domain essential for the function of heteromeric polycystin complexes. EMBO J. 2010;29:1176-91 pubmed publisher
    Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in two genes, PKD1 and PKD2, which encode polycystin-1 (PC1) and polycystin-2 (PC2), respectively...
  4. Bisgrove B, Snarr B, Emrazian A, Yost H. Polaris and Polycystin-2 in dorsal forerunner cells and Kupffer's vesicle are required for specification of the zebrafish left-right axis. Dev Biol. 2005;287:274-88 pubmed
    ..Two genes proposed to be important in this cilia-mediated signaling cascade are polaris and polycystin-2 (pkd2). Polaris is involved in ciliary assembly, while Pkd2 is proposed to function as a Ca(2+)-permeable cation channel...
  5. Streets A, Moon D, Kane M, Obara T, Ong A. Identification of an N-terminal glycogen synthase kinase 3 phosphorylation site which regulates the functional localization of polycystin-2 in vivo and in vitro. Hum Mol Genet. 2006;15:1465-73 pubmed
    b>PKD2 is mutated in 15% of patients with autosomal dominant polycystic kidney disease. Polycystin-2 (PC2), the PKD2 protein, is a non-selective Ca(2+)-permeable cation channel which may function at the cell surface and ER...
  6. Schottenfeld J, Sullivan Brown J, Burdine R. Zebrafish curly up encodes a Pkd2 ortholog that restricts left-side-specific expression of southpaw. Development. 2007;134:1605-15 pubmed
    ..ortholog of polycystic kidney disease 2, a gene that encodes the Ca(2+)-activated non-specific cation channel, Polycystin 2. We have characterized two alleles of cup, both of which display defects in organ positioning that resemble ..
  7. Fu X, Wang Y, Schetle N, Gao H, Pütz M, von Gersdorff G, et al. The subcellular localization of TRPP2 modulates its function. J Am Soc Nephrol. 2008;19:1342-51 pubmed publisher
    b>TRPP2, also known as polycystin-2, is a calcium permeable nonselective cation channel that is mutated in autosomal dominant polycystic kidney disease but has also been implicated in the regulation of cardiac development, renal tubular ..
  8. Fogelgren B, Lin S, Zuo X, Jaffe K, Park K, Reichert R, et al. The exocyst protein Sec10 interacts with Polycystin-2 and knockdown causes PKD-phenotypes. PLoS Genet. 2011;7:e1001361 pubmed publisher
    ..Mutations in PKD1 and PKD2, encoding polycystins-1 and -2, cause ADPKD...
  9. Obara T, Mangos S, Liu Y, Zhao J, Wiessner S, Kramer Zucker A, et al. Polycystin-2 immunolocalization and function in zebrafish. J Am Soc Nephrol. 2006;17:2706-18 pubmed
    ..b>pkd2 mRNA is ubiquitously expressed, and its presence in the larval kidney could be confirmed by reverse transcription-..

More Information


  1. Yuan S, Sun Z. Microinjection of mRNA and morpholino antisense oligonucleotides in zebrafish embryos. J Vis Exp. 2009;: pubmed publisher to prepare and microinject in vitro synthesized EGFP mRNA and a translational-blocking morpholino oligo against pkd2, a gene associated with autosomal dominant polycystic kidney disease (ADPKD), into 1-cell stage zebrafish embryos...
  2. Chang M, Lu J, Tian Y, Chen Y, Hung C, Huang Y, et al. Inhibition of the P2X7 receptor reduces cystogenesis in PKD. J Am Soc Nephrol. 2011;22:1696-706 pubmed publisher
    ..receptor agonist on cyst development in a zebrafish model of polycystic kidney disease in which we knocked down pkd2 by morpholinos...
  3. Astone M, Pizzi M, Peron M, Domenichini A, Guzzardo V, Töchterle S, et al. A GFP-Tagged Gross Deletion on Chromosome 1 Causes Malignant Peripheral Nerve Sheath Tumors and Carcinomas in Zebrafish. PLoS ONE. 2015;10:e0145178 pubmed publisher
  4. Sussman C, Ward C, Leightner A, Smith J, Agarwal R, Harris P, et al. Phosphodiesterase 1A modulates cystogenesis in zebrafish. J Am Soc Nephrol. 2014;25:2222-30 pubmed publisher
    ..Additionally, MO depletion of PDE1A aggravated phenotypes in pkd2 morphants, causing more severe body curvature, and human PDE1A RNA partially rescued pkd2 morphant phenotypes, ..
  5. Vasilyev A, Liu Y, Mudumana S, Mangos S, Lam P, Majumdar A, et al. Collective cell migration drives morphogenesis of the kidney nephron. PLoS Biol. 2009;7:e9 pubmed publisher
    ..Our results establish intimate links between nephron function, fluid flow, and morphogenesis. ..
  6. Gao H, Wang Y, Wegierski T, Skouloudaki K, P tz M, Fu X, et al. PRKCSH/80K-H, the protein mutated in polycystic liver disease, protects polycystin-2/TRPP2 against HERP-mediated degradation. Hum Mol Genet. 2010;19:16-24 pubmed publisher
    ..Identical phenotypic changes are induced by depletion or over-expression of TRPP2. Increased PRKCSH levels ameliorate developmental abnormalities caused by over-expressed TRPP2, whereas excess ..
  7. Chen J, van Eeden F, Warren K, Chin A, Nusslein Volhard C, Haffter P, et al. Left-right pattern of cardiac BMP4 may drive asymmetry of the heart in zebrafish. Development. 1997;124:4373-82 pubmed
    ..Thus, the pattern of cardiac BMP4 appears to be in the pathway by which the heart interprets lateralizing signals from the midline. ..
  8. Zhang M, Zhang J, Lin S, Meng A. ?-Catenin 1 and ?-catenin 2 play similar and distinct roles in left-right asymmetric development of zebrafish embryos. Development. 2012;139:2009-19 pubmed publisher
    ..Taken together, our findings suggest that ctnnb1 and ctnnb2 regulate multiple processes of laterality development in zebrafish embryos through similar and distinct mechanisms. ..
  9. Chang M, Ma T, Hung C, Tian Y, Chen Y, Yang C, et al. Metformin Inhibits Cyst Formation in a Zebrafish Model of Polycystin-2 Deficiency. Sci Rep. 2017;7:7161 pubmed publisher
    Autosomal dominant polycystic kidney disease (ADPKD) is a common kidney disease caused by mutations in PKD1 or PKD2. Metformin reduces cyst growth in mouse models of PKD1...
  10. Feng S, Okenka G, Bai C, Streets A, Newby L, DeChant B, et al. Identification and functional characterization of an N-terminal oligomerization domain for polycystin-2. J Biol Chem. 2008;283:28471-9 pubmed publisher
    ..The PKD2 protein, polycystin-2 (PC2 or TRPP2), is a member of the transient receptor potential (TRP) superfamily and functions as a non-selective calcium ..
  11. Bouvrette D, Sittaramane V, Heidel J, Chandrasekhar A, Bryda E. Knockdown of bicaudal C in zebrafish (Danio rerio) causes cystic kidneys: a nonmammalian model of polycystic kidney disease. Comp Med. 2010;60:96-106 pubmed
    ..These results demonstrate that the function of Bicc1 in the kidney is evolutionarily conserved, thus supporting the use of zebrafish as an alternative in vivo model to study the role of mammalian Bicc1 in renal cyst formation. ..
  12. Just S, Berger I, Meder B, Backs J, Keller A, Marquart S, et al. Protein kinase D2 controls cardiac valve formation in zebrafish by regulating histone deacetylase 5 activity. Circulation. 2011;124:324-34 pubmed publisher
    ..positional cloning that the bng phenotype is caused by a missense mutation (Y849N) in zebrafish protein kinase D2 (pkd2)...
  13. Rothschild S, Francescatto L, Drummond I, Tombes R. CaMK-II is a PKD2 target that promotes pronephric kidney development and stabilizes cilia. Development. 2011;138:3387-97 pubmed publisher
    ..Autosomal dominant polycystic kidney disease has been linked to mutations in the Ca²?-conducting TRP family member PKD2, the suppression of which in vertebrate model organisms results in kidney cysts...
  14. Cao Y, Semanchik N, Lee S, Somlo S, Barbano P, Coifman R, et al. Chemical modifier screen identifies HDAC inhibitors as suppressors of PKD models. Proc Natl Acad Sci U S A. 2009;106:21819-24 pubmed publisher
    ..that interact with ciliary signals, we performed a chemical modifier screen for the two phenotypes using zebrafish pkd2(hi4166) and ift172(hi2211) models...
  15. AbouAlaiwi W, Muntean B, Ratnam S, Joe B, Liu L, Booth R, et al. Survivin-induced abnormal ploidy contributes to cystic kidney and aneurysm formation. Circulation. 2014;129:660-72 pubmed publisher
    ..The present studies aim to examine whether and how cilia function (Pkd1 or Pkd2) and structure (Tg737) play a role in cystic kidney and aneurysm through survivin downregulation...
  16. Hurd T, Zhou W, Jenkins P, Liu C, Swaroop A, Khanna H, et al. The retinitis pigmentosa protein RP2 interacts with polycystin 2 and regulates cilia-mediated vertebrate development. Hum Mol Genet. 2010;19:4330-44 pubmed publisher that RP2 forms a calcium-sensitive complex with the autosomal dominant polycystic kidney disease protein polycystin 2. Ablation of RP2 by shRNA promotes swelling of the cilia tip that may be a result of aberrant trafficking of ..
  17. Mangos S, Lam P, Zhao A, Liu Y, Mudumana S, Vasilyev A, et al. The ADPKD genes pkd1a/b and pkd2 regulate extracellular matrix formation. Dis Model Mech. 2010;3:354-65 pubmed publisher
    ..Dorsal axis curvature was identical to the axis defects observed in pkd2 knockdown embryos. Combined pkd1a/b, pkd2 knockdown demonstrated that these genes interact in axial morphogenesis...
  18. Yuan S, Zhao L, Brueckner M, Sun Z. Intraciliary calcium oscillations initiate vertebrate left-right asymmetry. Curr Biol. 2015;25:556-67 pubmed publisher
    ..Previous work has indicated that sensory cilia and polycystin-2 (Pkd2), a cation channel, are required for sensing ciliary motility, yet their function and the molecular mechanism ..
  19. Heckel E, Boselli F, Roth S, Krudewig A, Belting H, Charvin G, et al. Oscillatory Flow Modulates Mechanosensitive klf2a Expression through trpv4 and trpp2 during Heart Valve Development. Curr Biol. 2015;25:1354-61 pubmed publisher
    ..We made similar observations for Trpp2, a known Trpv4 partner, and show that both the absence of Trpv4 or Trpp2 leads to valve defects...
  20. Roxo Rosa M, Jacinto R, Sampaio P, Lopes S. The zebrafish Kupffer's vesicle as a model system for the molecular mechanisms by which the lack of Polycystin-2 leads to stimulation of CFTR. Biol Open. 2015;4:1356-66 pubmed publisher
    ..Favouring its use, KV volume can be easily determined by in vivo imaging offering a live readout for screening compounds and genes that may prevent cyst enlargement through CFTR inhibition. ..
  21. Köttgen M, Buchholz B, Garcia Gonzalez M, Kotsis F, Fu X, Doerken M, et al. TRPP2 and TRPV4 form a polymodal sensory channel complex. J Cell Biol. 2008;182:437-47 pubmed publisher
    ..Polycystin-2 (TRPP2), an ion channel mutated in polycystic kidney disease, is required for cilia-mediated calcium transients but lacks ..
  22. Hu J, Barr M. ATP-2 interacts with the PLAT domain of LOV-1 and is involved in Caenorhabditis elegans polycystin signaling. Mol Biol Cell. 2005;16:458-69 pubmed
    ..ADPKD is caused by mutations in the polycystic kidney disease (PKD)1 or PKD2 gene, encoding polycystin (PC)-1 or PC-2, respectively. The C...
  23. Francescatto L, Rothschild S, Myers A, Tombes R. The activation of membrane targeted CaMK-II in the zebrafish Kupffer's vesicle is required for left-right asymmetry. Development. 2010;137:2753-62 pubmed publisher
    ..Left-sided CaMK-II activation was most dependent on an intact KV, the PKD2 Ca(2+) channel and gamma1 CaMK-II; however, alphaKAP, beta2 CaMK-II and the RyR3 ryanodine receptor were also ..
  24. Zheng W, Shen F, Hu R, Roy B, Yang J, Wang Q, et al. Far Upstream Element-Binding Protein 1 Binds the 3' Untranslated Region of PKD2 and Suppresses Its Translation. J Am Soc Nephrol. 2016;27:2645-57 pubmed publisher
    ..recapitulated in animal models by gene mutations in or dosage alterations of polycystic kidney disease 1 (PKD1) or PKD2, demonstrating that too much and too little PKD1/PKD2 are both pathogenic...