PKD2

Summary

Gene Symbol: PKD2
Description: polycystin 2, transient receptor potential cation channel
Alias: APKD2, PC2, PKD4, Pc-2, TRPP2, polycystin-2, autosomal dominant polycystic kidney disease type II protein, polycystic kidney disease 2 (autosomal dominant), transient receptor potential cation channel subfamily P member 2
Species: human
Products:     PKD2

Top Publications

  1. Mochizuki T, Wu G, Hayashi T, Xenophontos S, Veldhuisen B, Saris J, et al. PKD2, a gene for polycystic kidney disease that encodes an integral membrane protein. Science. 1996;272:1339-42 pubmed
    ..Nonsense mutations in this gene (PKD2) segregated with the disease in three PKD2 families...
  2. Barua M, Cil O, Paterson A, Wang K, He N, Dicks E, et al. Family history of renal disease severity predicts the mutated gene in ADPKD. J Am Soc Nephrol. 2009;20:1833-8 pubmed publisher
    Mutations of PKD1 and PKD2 account for 85 and 15% of cases of autosomal dominant polycystic kidney disease (ADPKD), respectively. Clinically, PKD1 is more severe than PKD2, with a median age at ESRD of 53.4 versus 72.7 yr...
  3. Li Q, Montalbetti N, Shen P, Dai X, Cheeseman C, Karpinski E, et al. Alpha-actinin associates with polycystin-2 and regulates its channel activity. Hum Mol Genet. 2005;14:1587-603 pubmed
    Polycystin-2 (PC2) is the product of the PKD2 gene, which is mutated in 10-15% patients of autosomal dominant polycystic kidney disease (ADPKD). PC2 is an integral transmembrane protein and acts as a calcium-permeable cation channel...
  4. Petri E, Celic A, Kennedy S, Ehrlich B, Boggon T, Hodsdon M. Structure of the EF-hand domain of polycystin-2 suggests a mechanism for Ca2+-dependent regulation of polycystin-2 channel activity. Proc Natl Acad Sci U S A. 2010;107:9176-81 pubmed publisher
    The C-terminal cytoplasmic tail of polycystin-2 (PC2/TRPP2), a Ca(2+)-permeable channel, is frequently mutated or truncated in autosomal dominant polycystic kidney disease...
  5. Koulen P, Cai Y, Geng L, Maeda Y, Nishimura S, Witzgall R, et al. Polycystin-2 is an intracellular calcium release channel. Nat Cell Biol. 2002;4:191-7 pubmed
  6. Montalbetti N, Li Q, Gonzalez Perrett S, Semprine J, Chen X, Cantiello H. Effect of hydro-osmotic pressure on polycystin-2 channel function in the human syncytiotrophoblast. Pflugers Arch. 2005;451:294-303 pubmed
    ..whose mutations cause autosomal dominant polycystic kidney disease is a transient receptor potential (TRP)-type (TRPP2) Ca(2+)-permeable, non-selective cation channel...
  7. Reynolds D, Hayashi T, Cai Y, Veldhuisen B, Watnick T, Lens X, et al. Aberrant splicing in the PKD2 gene as a cause of polycystic kidney disease. J Am Soc Nephrol. 1999;10:2342-51 pubmed
    ..that approximately 15% of families with autosomal dominant polycystic kidney disease (ADPKD) have mutations in PKD2. Identification of these mutations is central to identifying functionally important regions of gene and to ..
  8. Jurczyk A, Gromley A, Redick S, San Agustin J, Witman G, Pazour G, et al. Pericentrin forms a complex with intraflagellar transport proteins and polycystin-2 and is required for primary cilia assembly. J Cell Biol. 2004;166:637-43 pubmed
    ..depletion by RNA interference disrupts basal body localization of IFT proteins and the cation channel polycystin-2 (PC2), and inhibits primary cilia assembly in human epithelial cells...
  9. Bertuccio C, Chapin H, Cai Y, Mistry K, Chauvet V, Somlo S, et al. Polycystin-1 C-terminal cleavage is modulated by polycystin-2 expression. J Biol Chem. 2009;284:21011-26 pubmed publisher
    ..This effect is independent of Ca2+ but is regulated by sequences contained within the PC-2 C-terminal tail, suggesting a mechanism through which PC-1 and PC-2 may modulate a novel signaling pathway. ..

More Information

Publications83

  1. Ong A, Harris P. Molecular pathogenesis of ADPKD: the polycystin complex gets complex. Kidney Int. 2005;67:1234-47 pubmed
    ..Following the identification of the first ADPKD gene, PKD1, 10 years ago and PKD2 2 years later, considerable progress has been made in defining the etiology and understanding the pathogenesis of ..
  2. Roitbak T, Surviladze Z, Tikkanen R, Wandinger Ness A. A polycystin multiprotein complex constitutes a cholesterol-containing signalling microdomain in human kidney epithelia. Biochem J. 2005;392:29-38 pubmed
  3. Grimm D, Karihaloo A, Cai Y, Somlo S, Cantley L, Caplan M. Polycystin-2 regulates proliferation and branching morphogenesis in kidney epithelial cells. J Biol Chem. 2006;281:137-44 pubmed
    ..Loss or mutation of polycystin-1 or polycystin-2, the respective proteins encoded by the ADPKD genes PKD1 and PKD2, is associated with most cases of ADPKD...
  4. Li X, Luo Y, Starremans P, McNamara C, Pei Y, Zhou J. Polycystin-1 and polycystin-2 regulate the cell cycle through the helix-loop-helix inhibitor Id2. Nat Cell Biol. 2005;7:1202-12 pubmed
    ..Increased cell proliferation is a key feature of the disease. Here, we show that the ADPKD protein polycystin-2 (PC2) regulates the cell cycle through direct interaction with Id2, a member of the helix-loop-helix (HLH) protein ..
  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. Celic A, Petri E, Demeler B, Ehrlich B, Boggon T. Domain mapping of the polycystin-2 C-terminal tail using de novo molecular modeling and biophysical analysis. J Biol Chem. 2008;283:28305-12 pubmed publisher
    In polycystic kidney disease (PKD), polycystin-2 (PC2) is frequently mutated or truncated in the C-terminal cytoplasmic tail (PC2-C)...
  7. 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 ..
  8. Park E, Sung Y, Yang M, Noh J, Park S, Lee T, et al. Cyst formation in kidney via B-Raf signaling in the PKD2 transgenic mice. J Biol Chem. 2009;284:7214-22 pubmed publisher
    ..autosomal dominant polycystic kidney disease (ADPKD) have been well known to include the mutational inactivation of PKD2. Although haploinsufficiency and loss of heterozygosity at the Pkd2 locus can cause cyst formation in mice, ..
  9. Qian F, Germino F, Cai Y, Zhang X, Somlo S, Germino G. PKD1 interacts with PKD2 through a probable coiled-coil domain. Nat Genet. 1997;16:179-83 pubmed
    ..C terminus of the PKD1 gene product, polycystin, and demonstrate that it binds specifically to the C terminus of PKD2. Homotypic interactions involving the C terminus of each are also demonstrated...
  10. Audrezet M, Cornec Le Gall E, Chen J, Redon S, Quere I, Creff J, et al. Autosomal dominant polycystic kidney disease: comprehensive mutation analysis of PKD1 and PKD2 in 700 unrelated patients. Hum Mutat. 2012;33:1239-50 pubmed publisher
    ..polycystic kidney disease (ADPKD), the most common inherited kidney disorder, is caused by mutations in PKD1 or PKD2. The molecular diagnosis of ADPKD is complicated by extensive allelic heterogeneity and particularly by the ..
  11. Gonzalez Perrett S, Kim K, Ibarra C, Damiano A, Zotta E, Batelli M, et al. Polycystin-2, the protein mutated in autosomal dominant polycystic kidney disease (ADPKD), is a Ca2+-permeable nonselective cation channel. Proc Natl Acad Sci U S A. 2001;98:1182-7 pubmed
    ..The polycystin-2 channel may be associated with fluid accumulation and/or ion transport regulation in target epithelia, including placenta. Dysregulation of this channel provides a mechanism for the onset and progression of ADPKD. ..
  12. Lehtonen S, Ora A, Olkkonen V, Geng L, Zerial M, Somlo S, et al. In vivo interaction of the adapter protein CD2-associated protein with the type 2 polycystic kidney disease protein, polycystin-2. J Biol Chem. 2000;275:32888-93 pubmed
    ..Such a function fits well with that hypothesized for the polycystin proteins in renal tubular epithelial cells, and the present findings suggest that CD2AP has a role in polycystin-2 function. ..
  13. Gallagher A, Cedzich A, Gretz N, Somlo S, Witzgall R. The polycystic kidney disease protein PKD2 interacts with Hax-1, a protein associated with the actin cytoskeleton. Proc Natl Acad Sci U S A. 2000;97:4017-22 pubmed
    Despite the recent positional cloning of the PKD1 and PKD2 genes, which are mutated in the great majority of patients with autosomal-dominant polycystic kidney disease (PKD), the pathogenic mechanism for cyst formation is still unclear...
  14. Li Q, Dai Y, Guo L, Liu Y, Hao C, Wu G, et al. Polycystin-2 associates with tropomyosin-1, an actin microfilament component. J Mol Biol. 2003;325:949-62 pubmed
    Polycystin-2 (PC2) is the product of the second cloned gene (PKD2) responsible for autosomal dominant polycystic kidney disease and has recently been shown to be a calcium-permeable cation channel...
  15. Schumann F, Hoffmeister H, Bader R, Schmidt M, Witzgall R, Kalbitzer H. Ca2+-dependent conformational changes in a C-terminal cytosolic domain of polycystin-2. J Biol Chem. 2009;284:24372-83 pubmed publisher
    The PKD1 and PKD2 genes are the genes that are mutated in patients suffering from autosomal dominant polycystic kidney disease...
  16. 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...
  17. Chen X, Li Q, Wu Y, Liang G, Lara C, Cantiello H. Submembraneous microtubule cytoskeleton: interaction of TRPP2 with the cell cytoskeleton. FEBS J. 2008;275:4675-83 pubmed publisher
    b>TRPP2, also called polycystin-2, the gene product of PKD2, is a membrane protein defective in 10-15% of cases of autosomal dominant polycystic kidney disease...
  18. Tsiokas L, Kim E, Arnould T, Sukhatme V, Walz G. Homo- and heterodimeric interactions between the gene products of PKD1 and PKD2. Proc Natl Acad Sci U S A. 1997;94:6965-70 pubmed
    PKD1 and PKD2 are two recently identified genes that are responsible for the vast majority of autosomal polycystic kidney disease, a common inherited disease that causes progressive renal failure...
  19. Veldhuisen B, Saris J, de Haij S, Hayashi T, Reynolds D, Mochizuki T, et al. A spectrum of mutations in the second gene for autosomal dominant polycystic kidney disease (PKD2). Am J Hum Genet. 1997;61:547-55 pubmed
    ..These findings suggest that the first step toward cyst formation in PKD2 patients is the loss of one functional copy of polycystin-2.
  20. Koptides M, Hadjimichael C, Koupepidou P, Pierides A, Constantinou Deltas C. Germinal and somatic mutations in the PKD2 gene of renal cysts in autosomal dominant polycystic kidney disease. Hum Mol Genet. 1999;8:509-13 pubmed
    ..is caused by mutations in one of three genes: PKD1 on chromosome 16 accounts for approximately 85% of cases whereas PKD2 on chromosome 4 accounts for approximately 15%. Mutations in the PKD3 gene are rare...
  21. Tsiokas L, Arnould T, Zhu C, Kim E, Walz G, Sukhatme V. Specific association of the gene product of PKD2 with the TRPC1 channel. Proc Natl Acad Sci U S A. 1999;96:3934-9 pubmed
    The function(s) of the genes (PKD1 and PKD2) responsible for the majority of cases of autosomal dominant polycystic kidney disease is unknown...
  22. Li Y, Wright J, Qian F, Germino G, Guggino W. Polycystin 2 interacts with type I inositol 1,4,5-trisphosphate receptor to modulate intracellular Ca2+ signaling. J Biol Chem. 2005;280:41298-306 pubmed
    ..polycystic kidney disease, a common cause of renal failure, arises from mutations in either the PKD1 or the PKD2 gene. The precise function of both PKD gene products polycystins (PCs) 1 and 2 remain controversial...
  23. Newby L, Streets A, Zhao Y, Harris P, Ward C, Ong A. Identification, characterization, and localization of a novel kidney polycystin-1-polycystin-2 complex. J Biol Chem. 2002;277:20763-73 pubmed
  24. Köttgen M, Benzing T, Simmen T, Tauber R, Buchholz B, Feliciangeli S, et al. Trafficking of TRPP2 by PACS proteins represents a novel mechanism of ion channel regulation. EMBO J. 2005;24:705-16 pubmed
    ..Furthermore, PACS proteins may represent a novel molecular mechanism for ion channel trafficking, directing acidic cluster-containing ion channels to distinct subcellular compartments. ..
  25. Gonzalez Perrett S, Batelli M, Kim K, Essafi M, Timpanaro G, Moltabetti N, et al. Voltage dependence and pH regulation of human polycystin-2-mediated cation channel activity. J Biol Chem. 2002;277:24959-66 pubmed
    Polycystin-2, the product of the human PKD2 gene, whose mutations cause autosomal dominant polycystic kidney disease, is a large conductance, Ca(2+)-permeable non-selective cation channel...
  26. Bergmann C, von Bothmer J, Ortiz Brüchle N, Venghaus A, Frank V, Fehrenbach H, et al. Mutations in multiple PKD genes may explain early and severe polycystic kidney disease. J Am Soc Nephrol. 2011;22:2047-56 pubmed publisher
    ..Autosomal dominant polycystic kidney disease (ADPKD) is typically a late-onset disease caused by mutations in PKD1 or PKD2, but about 2% of patients with ADPKD show an early and severe phenotype that can be clinically indistinguishable ..
  27. Neumann H, Bacher J, Nabulsi Z, Ortiz Brüchle N, Hoffmann M, Schaeffner E, et al. Adult patients with sporadic polycystic kidney disease: the importance of screening for mutations in the PKD1 and PKD2 genes. Int Urol Nephrol. 2012;44:1753-62 pubmed publisher
    ..Family history was evaluated regarding ADPKD. Germline mutation screening of the PKD1 and PKD2 genes was performed for intragenic mutations and for large deletions...
  28. Magistroni R, He N, Wang K, Andrew R, Johnson A, Gabow P, et al. Genotype-renal function correlation in type 2 autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 2003;14:1164-74 pubmed
    ..Mutations of two genes, PKD1 and PKD2, account for the disease in approximately 80 to 85% and 10 to 15% of the cases, respectively...
  29. Chauvet V, Qian F, Boute N, Cai Y, Phakdeekitacharoen B, Onuchic L, et al. Expression of PKD1 and PKD2 transcripts and proteins in human embryo and during normal kidney development. Am J Pathol. 2002;160:973-83 pubmed
    ..Most cases result from mutations of PKD1 or PKD2 encoding polycystin-1 or polycystin-2, respectively...
  30. Wu Y, Dai X, Li Q, Chen C, Mai W, Hussain Z, et al. Kinesin-2 mediates physical and functional interactions between polycystin-2 and fibrocystin. Hum Mol Genet. 2006;15:3280-92 pubmed
    Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in PKD1, encoding polycystin-1 (PC1), or PKD2 (polycystin-2, PC2)...
  31. Liang G, Yang J, Wang Z, Li Q, Tang Y, Chen X. Polycystin-2 down-regulates cell proliferation via promoting PERK-dependent phosphorylation of eIF2alpha. Hum Mol Genet. 2008;17:3254-62 pubmed publisher
    ..Polycystin-2 (PC2), in which mutations account for 10-15% of ADPKD, was previously shown to down-regulate cell proliferation, but the ..
  32. Li Q, Shen P, Wu G, Chen X. Polycystin-2 interacts with troponin I, an angiogenesis inhibitor. Biochemistry. 2003;42:450-7 pubmed
    Polycystin-2 (PC2), encoded by the PKD2 gene, is mutated in 10-15% of autosomal dominant polycystic kidney disease (ADPKD) patients. PC2 is a Ca(2+)-permeable nonselective cation channel and is present in kidney and many other organs...
  33. Gloi A, Buchanan R. Dosimetric assessment of prostate cancer patients through principal component analysis (PCA). J Appl Clin Med Phys. 2013;14:3882 pubmed publisher
    ..5% of the variance in their shapes. The urethra DVHs are described by two principal components, PC1 and PC2, which account for 94.98% and 3.15% of the variance, respectively...
  34. Azoitei N, Cobbaut M, Becher A, Van Lint J, Seufferlein T. Protein kinase D2: a versatile player in cancer biology. Oncogene. 2018;37:1263-1278 pubmed publisher
    Protein kinase D2 (PKD2) is a serine/threonine kinase that belongs to the PKD family of calcium-calmodulin kinases, which comprises three isoforms: PKD1, PKD2, and PKD3...
  35. Schneider M, Rodriguez A, Nomura H, Zhou J, Morton C, Reeders S, et al. A gene similar to PKD1 maps to chromosome 4q22: a candidate gene for PKD2. Genomics. 1996;38:1-4 pubmed
    ..Finally, in situ hybridization studies localize this novel gene to 4q22, where PKD2, the second most common locus for ADPKD, is known to map. Therefore, R48321 is an excellent candidate gene for PKD2. ..
  36. Noshadi M, Ghafourian A. Groundwater quality analysis using multivariate statistical techniques (case study: Fars province, Iran). Environ Monit Assess. 2016;188:419 pubmed publisher
    ..Therefore, the first dominant factor was salinity. In PC2, HCO3 and pH were the dominant parameters, which may indicate weathering of silicate minerals...
  37. Majed L, Heugas A, Siegler I. Changes in movement organization and control strategies when learning a biomechanically constrained gait pattern, racewalking: a PCA study. Exp Brain Res. 2017;235:931-940 pubmed publisher
    ..explaining an upper-extremities/antero-posterior component (PC1) and a lower-extremities/vertical component (PC2)...
  38. Ko J. Functional Study of the Primary Cilia in ADPKD. Adv Exp Med Biol. 2016;933:45-57 pubmed
    ..Recent studies have indicated that the disruption of ciliary proteins including polycystin-1 (PC1), polycystin-2 (PC2), and members of the intraflagellar transport (IFT) family induce the development of polycystic kidney disease (PKD)..
  39. Lee E. Cell Proliferation and Apoptosis in ADPKD. Adv Exp Med Biol. 2016;933:25-34 pubmed
    ..With disruption of either PKD1 or PKD2, the main causative genes of ADPKD, intracellular calcium homeostasis and cAMP accumulation are disrupted, which in ..
  40. Mou R, Cao Z, Lin X, Wu L, Cao Z, Zhu Z, et al. Characterization of the phytochelatins and their derivatives in rice exposed to cadmium based on high-performance liquid chromatography coupled with data-dependent hybrid linear ion trap orbitrap mass spectrometry. Rapid Commun Mass Spectrom. 2016;30:1891-900 pubmed publisher
    ..Among them, seven PC derivatives, i.e., iso-PC2 (Asn), iso-PC3 (Asn), iso-PC2 (Cys), des-?Glu-iso-PC3 (Ser), des-Cys-iso-PC2 (Glu), des-Cys-iso-PC3 (Glu) and des-..
  41. Li Y, Santoso N, Yu S, Woodward O, Qian F, Guggino W. Polycystin-1 interacts with inositol 1,4,5-trisphosphate receptor to modulate intracellular Ca2+ signaling with implications for polycystic kidney disease. J Biol Chem. 2009;284:36431-41 pubmed publisher
    The PKD1 or PKD2 genes encode polycystins (PC) 1 and 2, which are associated with polycystic kidney disease...
  42. Losekoot M, Ruivenkamp C, Tholens A, Grimbergen J, Vijfhuizen L, Vermeer S, et al. Neonatal onset autosomal dominant polycystic kidney disease (ADPKD) in a patient homozygous for a PKD2 missense mutation due to uniparental disomy. J Med Genet. 2012;49:37-40 pubmed publisher
    Autosomal dominant polycystic kidney disease (ADPKD), due to a heterozygous mutation in PKD1 or PKD2, is usually an adult onset disease. Renal cystic disease is generally milder in PKD2 patients than in PKD1 patients...
  43. Ren J, Xia H, Yang J, Zhu J, Zhang W, Chen G, et al. Down-regulation of polycystin in lymphatic malformations: possible role in the proliferation of lymphatic endothelial cells. Hum Pathol. 2017;65:231-238 pubmed publisher
    ..Recent studies have demonstrated that the mutant embryos of PKD1 and PKD2, encoding polycystin-1 (PC-1) and polycystin-2 (PC-2), respectively, result in aberrant lymphatic vessels similar ..
  44. Zhang P, Luo Y, Chasan B, Gonzalez Perrett S, Montalbetti N, Timpanaro G, et al. The multimeric structure of polycystin-2 (TRPP2): structural-functional correlates of homo- and hetero-multimers with TRPC1. Hum Mol Genet. 2009;18:1238-51 pubmed publisher
    Polycystin-2 (PC2, TRPP2), the gene product of PKD2, whose mutations cause autosomal dominant polycystic kidney disease (ADPKD), belongs to the superfamily of TRP channels...
  45. Bataille S, Demoulin N, Devuyst O, Audrezet M, Dahan K, Godin M, et al. Association of PKD2 (polycystin 2) mutations with left-right laterality defects. Am J Kidney Dis. 2011;58:456-60 pubmed publisher
    Mutations in the PKD1 (polycystin 1) and PKD2 (polycystin 2) genes cause autosomal dominant polycystic kidney disease (ADPKD). Most Pkd2-null mouse embryos present with left-right laterality defects...
  46. Hofherr A, Wagner C, Watnick T, Köttgen M. Targeted rescue of a polycystic kidney disease mutation by lysosomal inhibition. Kidney Int. 2016;89:949-55 pubmed publisher
    ..Here we show that the ADPKD missense variant TRPP2(D511V) greatly reduces TRPP2 protein stability, and that TRPP2(D511V) function can be rescued in vivo by small ..
  47. Yang Y, Hodsdon M, Lolis E, Ehrlich B. Conformational dynamics of Ca2+-dependent responses in the polycystin-2 C-terminal tail. Biochem J. 2016;473:285-96 pubmed publisher
    b>PC2 (polycystin-2) forms a Ca(2+)-permeable channel in the cell membrane and its function is regulated by cytosolic Ca(2+) levels...
  48. Hajarnis S, Patel V, Aboudehen K, Attanasio M, Cobo Stark P, Pontoglio M, et al. Transcription Factor Hepatocyte Nuclear Factor-1β (HNF-1β) Regulates MicroRNA-200 Expression through a Long Noncoding RNA. J Biol Chem. 2015;290:24793-805 pubmed publisher
    ..and previous studies have shown that HNF-1β regulates the transcription of cystic disease genes, including Pkd2 and Pkhd1...
  49. Nigro E, Castelli M, Boletta A. Role of the Polycystins in Cell Migration, Polarity, and Tissue Morphogenesis. Cells. 2015;4:687-705 pubmed publisher
    ..We highlight here several open questions and controversies. Finally, we try to outline some of the next steps required to study these processes and their relevance in physiological and pathological conditions. ..
  50. Jorsal T, Rhee N, Pedersen J, Wahlgren C, Mortensen B, Jepsen S, et al. Enteroendocrine K and L cells in healthy and type 2 diabetic individuals. Diabetologia. 2018;61:284-294 pubmed publisher
    ..peptide-1, glucose-dependent insulinotropic polypeptide, peptide YY, prohormone convertase (PC) 1/3 and PC2 were observed along the intestinal tract...
  51. Hama T, Park F. Heterotrimeric G protein signaling in polycystic kidney disease. Physiol Genomics. 2016;48:429-45 pubmed publisher
    ..cells caused by naturally occurring mutations in two distinct genes, polycystic kidney disease 1 (PKD1) and 2 (PKD2)...
  52. Chen J, Lu L, Feng Y, Wang H, Dai L, Li Y, et al. PKD2 mediates multi-drug resistance in breast cancer cells through modulation of P-glycoprotein expression. Cancer Lett. 2011;300:48-56 pubmed publisher
    ..Herein, we have identified protein kinase D isoform 2 (PKD2) as an important regulator of MDR and P-gp expression in paclitaxel-treated breast cancer cell lines...
  53. Jerman S, Ward H, Lee R, Lopes C, Fry A, Macdougall M, et al. OFD1 and flotillins are integral components of a ciliary signaling protein complex organized by polycystins in renal epithelia and odontoblasts. PLoS ONE. 2014;9:e106330 pubmed publisher
    ..Together, these data suggest that polycystins are necessary for assembly of a novel flotillin-containing ciliary signaling complex and provide a molecular rationale for the common renal pathologies caused by OFD1 and PKD mutations. ..
  54. Wu K, Shen B, Jiang F, Xia L, Fan T, Qin M, et al. TRPP2 Enhances Metastasis by Regulating Epithelial-Mesenchymal Transition in Laryngeal Squamous Cell Carcinoma. Cell Physiol Biochem. 2016;39:2203-2215 pubmed
    ..b>TRPP2, a nonselective cation channel, is expressed in various cell types and participates in many biological processes...
  55. Zhang W, Zhang D, Wu Y, Sun T, Mei C. [Gene diagnosis of autosomal dominant polycystic kidney disease type 2 using microsatellite DNA tightly linked to polycystic kidney disease gene 2]. Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2004;21:325-8 pubmed
    ..Three families were found to be linked to PKD2 in 20 families. Two carriers of PKD2 mutation were revealed by linkage analysis...
  56. Woo Y, Kim D, Koo N, Kim Y, Lee S, Ko J, et al. Profiling of miRNAs and target genes related to cystogenesis in ADPKD mouse models. Sci Rep. 2017;7:14151 pubmed publisher
    ..cystogenesis through integrative analysis of miRNA- and RNA-seq using two ADPKD mouse models (conditional Pkd1- or Pkd2-deficient mice), at three different time points (P1, P3, and P7)...
  57. Zhao Y, Ali A, Yan E. The plant economics spectrum is structured by leaf habits and growth forms across subtropical species. Tree Physiol. 2017;37:173-185 pubmed publisher
    ..clustered at other end in each of the axes of the leaf and wood syndromes (PC1-axis) and the plant height strategy (PC2-axis). The local WPES, LES and WES were tightly correlated with each other...
  58. Ma M, Gallagher A, Somlo S. Ciliary Mechanisms of Cyst Formation in Polycystic Kidney Disease. Cold Spring Harb Perspect Biol. 2017;9: pubmed publisher
    ..The causal genes PKD1 and PKD2 encode transmembrane proteins polycystin 1 (PC1) and polycystin 2 (PC2), respectively...
  59. de Almeida R, Clendenon S, Richards W, Boedigheimer M, Damore M, Rossetti S, et al. Transcriptome analysis reveals manifold mechanisms of cyst development in ADPKD. Hum Genomics. 2016;10:37 pubmed
    ..Transcriptogram analysis identifies novel pathways altered in ADPKD, providing new avenues to identify both ADPKD's mechanisms of pathogenesis and pharmaceutical targets to ameliorate the progression of the disease. ..
  60. McGahon M, Fernandez J, Dash D, McKee J, Simpson D, Zholos A, et al. TRPV2 Channels Contribute to Stretch-Activated Cation Currents and Myogenic Constriction in Retinal Arterioles. Invest Ophthalmol Vis Sci. 2016;57:5637-5647 pubmed publisher
    Activation of the transient receptor potential channels, TRPC6, TRPM4, and TRPP1 (PKD2), has been shown to contribute to the myogenic constriction of cerebral arteries...
  61. Jin M, Xie Y, Chen Z, Liao Y, Li Z, Hu P, et al. System analysis of gene mutations and clinical phenotype in Chinese patients with autosomal-dominant polycystic kidney disease. Sci Rep. 2016;6:35945 pubmed publisher
    ..polycystic kidney disease (ADPKD) is the most common inherited kidney disorder mainly caused by mutation in PKD1/PKD2. However, ethnic differences in mutations, the association between mutation genotype/clinical phenotype, and the ..
  62. Montalbetti N, Li Q, Wu Y, Chen X, Cantiello H. Polycystin-2 cation channel function in the human syncytiotrophoblast is regulated by microtubular structures. J Physiol. 2007;579:717-28 pubmed
    ..PC2, a TRP-type (TRPP2) non-selective cation channel, is present in primary cilia of renal epithelial cells, a microtubule-based ..
  63. Hayashi T, Mochizuki T, Reynolds D, Wu G, Cai Y, Somlo S. Characterization of the exon structure of the polycystic kidney disease 2 gene (PKD2). Genomics. 1997;44:131-6 pubmed
    b>PKD2, the gene defective in the second form of autosomal dominant polycystic kidney disease (ADPKD), has been identified by positional cloning and found to encode an integral membrane protein with similarity to the gene for the more ..
  64. Kim B, Park E, Yoo K, Choi K, Kim Y, Seong J, et al. N-myc downstream-regulated gene 1 is involved in the regulation of cystogenesis in transgenic mice overexpressing human PKD2 gene. Proteomics. 2013;13:134-41 pubmed publisher
    ..In a previous study, transgenic mice overexpressing human PKD2 gene were produced as an ADPKD animal model...
  65. Obeidova L, Elisáková V, Stekrova J, Reiterova J, Merta M, Tesar V, et al. Novel mutations of PKD genes in the Czech population with autosomal dominant polycystic kidney disease. BMC Med Genet. 2014;15:41 pubmed publisher
    ..ADPKD) is the most common hereditary renal disorder caused by mutation in either one of two genes, PKD1 and PKD2. High structural and sequence complexity of PKD genes makes the mutational diagnostics of ADPKD challenging...
  66. Tang W, Yuan Q, Xu B, Osei K, Wang J. Exenatide substantially improves proinsulin conversion and cell survival that augment Ins2+/Akita beta cell function. Mol Cell Endocrinol. 2017;439:297-307 pubmed publisher
    ..due to enhanced conversion of proinsulin to insulin, having nothing to do with the prohormone convertase PC1/3 and PC2 levels. The 15 min improvement is calcium-independent...
  67. Li Q, Liu Y, Shen P, Dai X, Wang S, Smillie L, et al. Troponin I binds polycystin-L and inhibits its calcium-induced channel activation. Biochemistry. 2003;42:7618-25 pubmed
    ..Taken together, this study suggests that troponin I acts as a regulatory subunit of the PCL channel complex and provides the first direct evidence that PCL is associated with the actin cytoskeleton through troponin I. ..
  68. Zhu P, Sieben C, Xu X, Harris P, Lin X. Autophagy activators suppress cystogenesis in an autosomal dominant polycystic kidney disease model. Hum Mol Genet. 2017;26:158-172 pubmed publisher
    Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in either PKD1 or PKD2. It is one of the most common heritable human diseases with eventual development of renal failure; however, effective treatment is lacking...
  69. Cornec Le Gall E, Audrezet M, Renaudineau E, Hourmant M, Charasse C, Michez E, et al. PKD2-Related Autosomal Dominant Polycystic Kidney Disease: Prevalence, Clinical Presentation, Mutation Spectrum, and Prognosis. Am J Kidney Dis. 2017;70:476-485 pubmed publisher
    b>PKD2-related autosomal dominant polycystic kidney disease (ADPKD) is widely acknowledged to be of milder severity than PKD1-related disease, but population-based studies depicting the exact burden of the disease are lacking...
  70. Mato E, Lucas M, Petriz J, Gomis R, Novials A. Identification of a pancreatic stellate cell population with properties of progenitor cells: new role for stellate cells in the pancreas. Biochem J. 2009;421:181-91 pubmed publisher
    ..islet amyloid polypeptide, glucagon, the glucose transporter GLUT2, chromogranin A and the convertases PC1/3 and PC2 were also detected...
  71. Chen J, Feng Y, Lu L, Wang H, Dai L, Li Y, et al. Interferon-?-induced PD-L1 surface expression on human oral squamous carcinoma via PKD2 signal pathway. Immunobiology. 2012;217:385-93 pubmed publisher
    ..Herein, we have identified protein kinase D isoform 2 (PKD2) as an important regulator of PD-L1 expression on human oral squamous carcinoma cells induced by IFN-?...
  72. Yang Y, Ye Y, Wang Y, Sun Y, Pan D, Cao J. Effect of high pressure treatment on metabolite profile of marinated meat in soy sauce. Food Chem. 2018;240:662-669 pubmed publisher
    ..PC1 and PC2 explained a total of 75.4 and 11.9% of variables, respectively...
  73. Omran A, Saternos H, Althobaiti Y, Wisner A, Sari Y, Nauli S, et al. Alcohol consumption impairs the ependymal cilia motility in the brain ventricles. Sci Rep. 2017;7:13652 pubmed publisher
    ..Thus, our studies reinforce the presence of three types of ependymal cilia in the brain ventricles and demonstrate the involvement of ethanol as a risk factor for the impairment of ependymal cilia motility in the brain. ..
  74. Xenophontos S, Constantinides R, Hayashi T, Mochizuki T, Somlo S, Pierides A, et al. A translation frameshift mutation induced by a cytosine insertion in the polycystic kidney disease 2 gene (PDK2). Hum Mol Genet. 1997;6:949-52 pubmed
    Mutations in the PKD2 gene on the long arm of chromosome 4 are responsible for approximately 15% of cases of polycystic kidney disease...