A polycystin-2 (TRPP2) dimerization domain essential for the function of heteromeric polycystin complexes

EMBO Journal

Volumn 29, Issue 7, 2010, Pages 1176-1191

  Giamarchi, Aurélie ^a   Feng, Shuang ^b   Rodat Despoix, Lise ^a   Xu, Yaoxian ^b   Bubenshchikova, Ekaterina ^c   Newby, Linda J ^b   Hao, Jizhe ^a   Gaudioso, Christelle ^a   Crest, Marcel ^a   Lupas, Andrei N ^d   Honoré, Eric ^e   Williamson, Michael P ^f   Obara, Tomoko ^c,g   Ong, Albert C M ^b   Delmas, Patrick ^a  

^a University of Mediterranée   (France)

^b UNIVERSITY OF SHEFFIELD   (United Kingdom)

^c METROHEALTH MEDICAL CENTER   (United States)

^d MAX PLANCK INSTITUTE FOR DEVELOPMENTAL BIOLOGY   (Germany)

^e INSTITUT DE PHARMACOLOGIE MOLÉCULAIRE ET CELLULAIRE   (France)

^f UNIVERSITY OF SHEFFIELD   (United Kingdom)

^g CASE WESTERN RESERVE UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

Ion channel signalling complex; Polycystic kidney disease; Polycystin 1; Polycystin 2; TRP channel

Indexed keywords

ADENOSINE TRIPHOSPHATE; AMILORIDE; CALCIUM ION; COMPLEMENTARY DNA; GREEN FLUORESCENT PROTEIN; ION CHANNEL; MEMBRANE RECEPTOR; MESSENGER RNA; POLYCYSTIN 1; POLYCYSTIN 2;

AMINO ACID SEQUENCE; ANIMAL EXPERIMENT; ARTICLE; AUTOSOMAL DOMINANT DISORDER; CALCIUM TRANSPORT; CELL MEMBRANE; CELL STRAIN HEK293; COMPLEX FORMATION; CONFOCAL MICROSCOPY; CONTROLLED STUDY; DIMERIZATION; ELECTRON MICROSCOPY; EMBRYO; ENDOPLASMIC RETICULUM; GENE MUTATION; HUMAN; HUMAN CELL; IMMUNOCYTOCHEMISTRY; KIDNEY POLYCYSTIC DISEASE; MOUSE; NONHUMAN; NUCLEOTIDE SEQUENCE; OLIGOMERIZATION; PRIORITY JOURNAL; PROTEIN LOCALIZATION; PROTEIN PROTEIN INTERACTION; REVERSE TRANSCRIPTION POLYMERASE CHAIN REACTION; SITE DIRECTED MUTAGENESIS; TWO HYBRID SYSTEM; ULTRACENTRIFUGATION; YEAST;

AMINO ACID SEQUENCE; ANIMALS; CALCIUM; CELL LINE; DIMERIZATION; ENDOPLASMIC RETICULUM; GENE EXPRESSION; HUMANS; KIDNEY; MOLECULAR SEQUENCE DATA; MUTATION; POLYCYSTIC KIDNEY, AUTOSOMAL DOMINANT; PROTEIN BINDING; PROTEIN STRUCTURE, TERTIARY; SEQUENCE ALIGNMENT; TRPP CATION CHANNELS; TWO-HYBRID SYSTEM TECHNIQUES; ZEBRAFISH;

DANIO RERIO;

EID: 77950536515     PISSN: 02614189     EISSN: 14602075     Source Type: Journal    
DOI: 10.1038/emboj.2010.18     Document Type: Article

References (84)

1. Anderson RG, Orci L, Brown MS, Garcia-Segura LM, Goldstein JL (1983) Ultrastructural analysis of crystalloid endoplasmic reticu-lum in UT-1 cells and its disappearance in response to cholesterol. J Cell Sci 63: 1-20 (Pubitemid 13020329)
2. Arnould T, Kim E, Tsiokas L, Jochimsen F, Gruning W, Chang JD, Walz G (1998) The polycystic kidney disease 1 gene product mediates protein kinase C alpha-dependent and c-Jun N-terminal kinase-dependent activation of the transcription factor AP-1. J Biol Chem 273: 6013-6018 (Pubitemid 28144677)
3. Bai CX, Giamarchi A, Rodat-Despoix L, Padilla F, Downs T, Tsiokas L, Delmas P (2008a) Formation of a new receptor-operated channel by heteromeric assembly of TRPP2 and TRPC1 subunits. EMBO Rep 9: 472-479 (Pubitemid 351627286)
4. Bai CX, Kim S, Li WP, Streets AJ, Ong AC, Tsiokas L (2008b) Activation of TRPP2 through mDia1-dependent voltage gating. EMBO J 27: 1345-1356 (Pubitemid 351655182)
5. Bhunia AK, Piontek K, Boletta A, Liu L, Qian F, Xu PN, Germino FJ, Germino GG (2002) PKD1 induces p21(waf1) and regulation of the cell cycle via direct activation of the JAK-STAT signaling pathway in a process requiring PKD2. Cell 109: 157-168 (Pubitemid 34518255)
6. Birnbaumer L, Boulay G, Brown D, Jiang M, Dietrich A, Mikoshiba K, Zhu X, Qin N (2000) Mechanism of capacitative Ca2+ entry (CCE): interaction between IP3 receptor and TRP links the internal calcium storage compartment to plasma membrane CCE channels. Recent Prog Horm Res 55: 127-161; discussion 161-1122 (Pubitemid 32094448)
7. Bisgrove BW, Snarr BS, Emrazian A, Yost HJ (2005) Polaris and polycystin-2 in dorsal forerunner cells and Kupffer's vesicle are required for specification of the zebrafish left-right axis. Dev Biol 287: 274-288 (Pubitemid 41636764)
8. Cai Y, Maeda Y, Cedzich A, Torres VE, Wu G, Hayashi T, Mochizuki T, Park JH, Witzgall R, Somlo S (1999) Identification and characterization of polycystin-2, the PKD2 gene product. J Biol Chem 274: 28557-28565 (Pubitemid 129504727)
9. Calvet JP, Grantham JJ (2001) The genetics and physiology of polycystic kidney disease. Semin Nephrol 21: 107-123 (Pubitemid 32230544)
10. Celic A, Petri ET, Demeler B, Ehrlich BE, Boggon TJ (2008) Domain mapping of the polycystin-2 C-terminal tail using de novo molecular modeling and biophysical analysis. J Biol Chem 283: 28305-28312
11. Damann N, Voets T, Nilius B (2008) TRPs in our senses. Curr Biol 18: R880-R889
12. Delmas P (2004) Polycystins: from mechanosensation to gene regulation. Cell 118: 145-148 (Pubitemid 38962571)
13. 2+ stores. Neuron 36: 787-790 (Pubitemid 35447851)
14. Delmas P, Nauli SM, Li X, Coste B, Osorio N, Crest M, Brown DA, Zhou J (2004) Gating of the polycystin ion channel signaling complex in neurons and kidney cells. FASEB J 18: 740-742
15. Delmas P, Nomura H, Li X, Lakkis M, Luo Y, Segal Y, Fernandez-Fernandez JM, Harris P, Frischauf AM, Brown DA, Zhou J (2002a) Constitutive activation of G-proteins by polycystin-1 is antagonized by polycystin-2. J Biol Chem 277: 11276-11283 (Pubitemid 34952891)
16. Delmas P, Wanaverbecq N, Abogadie FC, Mistry M, Brown DA (2002b) Signaling microdomains define the specificity of receptor-mediated InsP(3) pathways in neurons. Neuron 34: 209-220 (Pubitemid 34442564)
17. Deng Y, Liu J, Zheng Q, Eliezer D, Kallenbach NR, Lu M (2006) Antiparallel four-stranded coiled coil specified by a 3-3-1 hydro-phobic heptad repeat. Structure 14: 247-255 (Pubitemid 43202014)
18. Feng S, Okenka GM, Bai CX, Streets AJ, Newby LJ, DeChant BT, Tsiokas L, Obara T, Ong AC (2008) Identification and functional characterization of an N-terminal oligomerization domain for polycystin-2. J Biol Chem 283: 28471-28479
19. Foggensteiner L, Bevan AP, Thomas R, Coleman N, Boulter C, Bradley J, Ibraghimov-Beskrovnaya O, Klinger K, Sandford R (2000) Cellular and subcellular distribution of polycystin-2, the protein product of the PKD2 gene. J Am Soc Nephrol 11: 814-827 (Pubitemid 30241715)
20. Fu X, Wang Y, Schetle N, Gao H, Putz M, von Gersdorff G, Walz G, Kramer-Zucker AG (2008) The subcellular localization of TRPP2 modulates its function. J Am Soc Nephrol 19: 1342-1351
21. Fujiwara Y, Minor Jr DL (2008) X-ray crystal structure of a TRPM assembly domain reveals an antiparallel four-stranded coiled-coil. J Mol Biol 383: 854-870
22. Gabow PA (1990) Autosomal dominant polycystic kidney disease-more than a renal disease. Am J Kidney Dis 16: 403-413
23. Geng L, Boehmerle W, Maeda Y, Okuhara DY, Tian X, Yu Z, Choe CU, Anyatonwu GI, Ehrlich BE, Somlo S (2008) Syntaxin 5 regulates the endoplasmic reticulum channel-release properties of polycystin-2. Proc Natl Acad Sci USA 105: 15920-15925
24. Geng L, Okuhara D, Yu Z, Tian X, Cai Y, Shibazaki S, Somlo S (2006) Polycystin-2 traffics to cilia independently of polycystin-1 by using an N-terminal RVxP motif. J Cell Sci 119: 1383-1395 (Pubitemid 43756554)
25. Giamarchi A, Padilla F, Coste B, Raoux M, Crest M, Honore E, Delmas P (2006) The versatile nature of the calcium-permeable cation channel TRPP2. EMBO Rep 7: 787-793 (Pubitemid 44275396)
26. Gonzalez-Perrett S, Kim K, Ibarra C, Damiano AE, Zotta E, Batelli M, Harris PC, Reisin IL, Arnaout MA, Cantiello HF (2001) Polycystin-2, the protein mutated in autosomal dominant poly-cystic kidney disease (ADPKD), is a Ca2+-permeable nonselec-tive cation channel. Proc Natl Acad Sci USA 98: 1182-1187 (Pubitemid 32121207)
27. Hanaoka K, Qian F, Boletta A, Bhunia AK, Piontek K, Tsiokas L, Sukhatme VP, Guggino WB, Germino GG (2000) Co-assembly of polycystin-1 and-2 produces unique cation-permeable currents. Nature 408: 990-994 (Pubitemid 32101647)
28. Harris PC (1999) Autosomal dominant polycystic kidney disease: clues to pathogenesis. Hum Mol Genet 8: 1861-1866 (Pubitemid 29458663)
29. Hughes J, Ward CJ, Peral B, Aspinwall R, Clark K, San Millan JL, Gamble V, Harris PC (1995) The polycystic kidney disease 1 (PKD1) gene encodes a novel protein with multiple cell recognition domains. Nat Genet 10: 151-160
30. Ishimaru Y, Inada H, Kubota M, Zhuang H, Tominaga M, Matsunami H (2006) Transient receptor potential family members PKD1L3 and PKD2L1 form a candidate sour taste receptor. Proc Natl Acad Sci USA 103: 12569-12574 (Pubitemid 44267299)
31. Jenke M, Sanchez A, Monje F, Stuhmer W, Weseloh RM, Pardo LA (2003) C-terminal domains implicated in the functional surface expression of potassium channels. EMBO J 22: 395-403 (Pubitemid 36193585)
32. Karcher C, Fischer A, Schweickert A, Bitzer E, Horie S, Witzgall R, Blum M (2005) Lack of a laterality phenotype in Pkd1 knock-out embryos correlates with absence of polycystin-1 in nodal cilia. Differentiation 73: 425-432 (Pubitemid 43920089)
33. Kim E, Arnould T, Sellin L, Benzing T, Comella N, Kocher O, Tsiokas L, Sukhatme VP, Walz G (1999) Interaction between RGS7 and polycystin. Proc Natl Acad Sci USA 96: 6371-6376 (Pubitemid 29256672)
34. + (SK(Ca)) channel from Rattus norvegicus. Proteins 70: 568-571
35. Kobori T, Smith GD, Sandford R, Edwardson JM (2009) The transient receptor potential (TRP) channels TRPP2 and TRPC1 form a heterotetramer with a 2:2 stoichiometry and an alternating subunit arrangement. J Biol Chem 284: 35507-35513
36. Köttgen M, Buchholz B, Garcia-Gonzalez MA, Kotsis F, Fu X, Doerken M, Boehlke C, Steffl D, Tauber R, Wegierski T, Nitschke R, Suzuki M, Kramer-Zucker A, Germino GG, Watnick T, Prenen J, Nilius B, Kuehn EW, Walz G (2008) TRPP2 and TRPV4 form a polymodal sensory channel complex. J Cell Biol 182: 437-447
37. Köttgen M, Walz G (2005) Subcellular localization and trafficking of polycystins. Pflugers Arch 451: 286-293 (Pubitemid 41424933)
38. Koulen P, Cai Y, Geng L, Maeda Y, Nishimura S, Witzgall R, Ehrlich BE, Somlo S (2002) Polycystin-2 is an intracellular calcium release channel. Nat Cell Biol 4: 191-197 (Pubitemid 34218192)
39. Li Q, Montalbetti N, Wu Y, Ramos A, Raychowdhury MK, Chen XZ, Cantiello HF (2006) Polycystin-2 cation channel function is under the control of microtubular structures in primary cilia of renal epithelial cells. J Biol Chem 281: 37566-37575 (Pubitemid 46042060)
40. Li Y, Wright JM, Qian F, Germino GG, Guggino WB (2005) Polycystin 2 interacts with type I inositol 1,4,5-trisphosphate receptor to modulate intracellular Ca2+ signaling. J Biol Chem 280: 41298-41306 (Pubitemid 41832186)
41. LopezJimenez ND, Cavenagh MM, Sainz E, Cruz-Ithier MA, Battey JF, Sullivan SL (2006) Two members of the TRPP family of ion channels, Pkd1l3 and Pkd2l1, are co-expressed in a subset of taste receptor cells. J Neurochem 98: 68-77 (Pubitemid 43882933)
42. Ludwig J, Owen D, Pongs O (1997) Carboxy-terminal domain mediates assembly of the voltage-gated rat ether-a-go-go potassium channel. EMBO J 16: 6337-6345 (Pubitemid 27483260)
43. Luik RM, Wang B, Prakriya M, Wu MM, Lewis RS (2008) Oligomerization of STIM1 couples ER calcium depletion to CRAC channel activation. Nature 454: 538-542
44. Luo Y, Vassilev PM, Li X, Kawanabe Y, Zhou J (2003) Native polycystin 2 functions as a plasma membrane Ca2+-permeable cation channel in renal epithelia. Mol Cell Biol 23: 2600-2607 (Pubitemid 36351038)
45. Lupas A, Van Dyke M, Stock J (1991) Predicting coiled coils from protein sequences. Science 252: 1162-1164 (Pubitemid 21917026)
46. Ma R, Li WP, Rundle D, Kong J, Akbarali HI, Tsiokas L (2005) PKD2 functions as an epidermal growth factor-activated plasma membrane channel. Mol Cell Biol 25: 8285-8298 (Pubitemid 41263017)
47. McGrath J, Somlo S, Makova S, Tian X, Brueckner M (2003) Two populations of node monocilia initiate left-right asymmetry in the mouse. Cell 114: 61-73 (Pubitemid 36859841)
48. Mochizuki T, Wu G, Hayashi T, Xenophontos SL, Veldhuisen B, Saris JJ, Reynolds DM, Cai Y, Gabow PA, Pierides A, Kimberling WJ, Breuning MH, Deltas CC, Peters DJ, Somlo S (1996) PKD2, a gene for polycystic kidney disease that encodes an integral membrane protein. Science 272: 1339-1342 (Pubitemid 26199975)
49. Nauli SM, Alenghat FJ, Luo Y, Williams E, Vassilev P, Li X, Elia AE, Lu W, Brown EM, Quinn SJ, Ingber DE, Zhou J (2003) Polycystins 1 and 2 mediate mechanosensation in the primary cilium of kidney cells. Nat Genet 33: 129-137 (Pubitemid 36177063)
50. Newby LJ, Streets AJ, Zhao Y, Harris PC, Ward CJ, Ong AC (2002) Identification, characterization, and localization of a novel kidney polycystin-1-polycystin-2 complex. J Biol Chem 277: 20763-20773 (Pubitemid 34967382)
51. Obara T, Mangos S, Liu Y, Zhao J, Wiessner S, Kramer-Zucker AG, Olale F, Schier AF, Drummond IA (2006) Polycystin-2 immuno-localization and function in zebrafish. J Am Soc Nephrol 17: 2706-2718
52. Ong AC, Harris PC (2005) Molecular pathogenesis of ADPKD: the polycystin complex gets complex. Kidney Int 67: 1234-1247
53. Ong AC, Harris PC, Davies DR, Pritchard L, Rossetti S, Biddolph S, Vaux DJ, Migone N, Ward CJ (1999a) Polycystin-1 expression in PKD1, early-onset PKD1, and TSC2/PKD1 cystic tissue. Kidney Int 56: 1324-1333 (Pubitemid 29451240)
54. Ong AC, Ward CJ, Butler RJ, Biddolph S, Bowker C, Torra R, Pei Y, Harris PC (1999b) Coordinate expression of the autosomal dominant polycystic kidney disease proteins, polycystin-2 and poly-cystin-1, in normal and cystic tissue. Am J Pathol 154: 1721-1729 (Pubitemid 29262854)
55. Parnell SC, Magenheimer BS, Maser RL, Zien CA, Frischauf AM, Calvet JP (2002) Polycystin-1 activation of c-Jun N-terminal kinase and AP-1 is mediated by heterotrimeric G proteins. J Biol Chem 277: 19566-19572 (Pubitemid 34967469)
56. Pennekamp P, Karcher C, Fischer A, Schweickert A, Skryabin B, Horst J, Blum M, Dworniczak B (2002) The ion channel poly-cystin-2 is required for left-right axis determination in mice. Curr Biol 12: 938-943 (Pubitemid 34689231)
57. Phelps CB, Gaudet R (2007) The role of the N terminus and transmembrane domain of TRPM8 in channel localization and tetramerization. J Biol Chem 282: 36474-36480
58. Praetorius HA, Spring KR (2003) Removal of the MDCK cell primary cilium abolishes flow sensing. J Membr Biol 191: 69-76 (Pubitemid 37011467)
59. Qian F, Germino FJ, Cai Y, Zhang X, Somlo S, Germino GG (1997) PKD1 interacts with PKD2 through a probable coiled-coil domain. Nat Genet 16: 179-183 (Pubitemid 27240617)
60. Roitbak T, Surviladze Z, Tikkanen R, Wandinger-Ness A (2005) A polycystin multiprotein complex constitutes a cholesterol-containing signalling microdomain in human kidney epithelia. Biochem J 392: 29-38 (Pubitemid 41689398)
61. Rossetti S, Consugar MB, Chapman AB, Torres VE, Guay-Woodford LM, Grantham JJ, Bennett WM, Meyers CM, Walker DL, Bae K, Zhang QJ, Thompson PA, Miller JP, Harris PC (2007) Comprehensive molecular diagnostics in autosomal dominant polycystic kidney disease. J Am Soc Nephrol 18: 2143-2160 (Pubitemid 47041080)
62. Sandford R, Sgotto B, Aparicio S, Brenner S, Vaudin M, Wilson RK, Chissoe S, Pepin K, Bateman A, Chothia C, Hughes J, Harris P (1997) Comparative analysis of the polycystic kidney disease 1 (PKD1) gene reveals an integral membrane glycoprotein with multiple evolutionary conserved domains. Hum Mol Genet 6: 1483-1489 (Pubitemid 27397024)
63. Scheffers MS, Le H, van der Bent P, Leonhard W, Prins F, Spruit L, Breuning MH, de Heer E, Peters DJ (2002) Distinct subcellular expression of endogenous polycystin-2 in the plasma membrane and Golgi apparatus of MDCK cells. Hum Mol Genet 11: 59-67 (Pubitemid 34100768)
64. Schmitt N, Schwarz M, Peretz A, Abitbol I, Attali B, Pongs O (2000) A recessive C-terminal Jervell and Lange-Nielsen mutation of the KCNQ1 channel impairs subunit assembly. EMBO J 19: 332-340 (Pubitemid 30072603)
65. Schottenfeld J, Sullivan-Brown J, Burdine RD (2007) Zebrafish curly up encodes a Pkd2 ortholog that restricts left-side-specific expression of southpaw. Development 134: 1605-1615
66. Schwake M, Jentsch TJ, Friedrich T (2003) A carboxy-terminal domain determines the subunit specificity of KCNQ K+ channel assembly. EMBO Rep 4: 76-81 (Pubitemid 36305382)
67. Sharif-Naeini R, Folgering JH, Bichet D, Duprat F, Lauritzen I, Arhatte M, Jodar M, Dedman A, Chatelain FC, Schulte U, Retailleau K, Loufrani L, Patel A, Sachs F, Delmas P, Peters DJ, Honoré E (2009) Polycystin-1 and-2 dosage regulates pressure sensing. Cell 139: 587-596
68. Streets AJ, Moon DJ, Kane ME, Obara T, Ong AC (2006) Identification of an N-terminal glycogen synthase kinase 3 phospho-rylation site which regulates the functional localization of polycystin-2 in vivo and in vitro. Hum Mol Genet 15: 1465-1473
69. Streets AJ, Wagner BE, Harris PC, Ward CJ, Ong AC (2009) Homophilic and heterophilic polycystin 1 interactions regulate E-cadherin recruitment and junction assembly in MDCK cells. J Cell Sci 122: 1410-1417
70. Sullivan-Brown J, Schottenfeld J, Okabe N, Hostetter CL, Serluca FC, Thiberge SY, Burdine RD (2008) Zebrafish mutations affecting cilia motility share similar cystic phenotypes and suggest a mechanism of cyst formation that differs from pkd2 morphants. Dev Biol 314: 261-275
71. Sun Z, Amsterdam A, Pazour GJ, Cole DG, Miller MS, Hopkins N (2004) A genetic screen in zebrafish identifies cilia genes as a principal cause of cystic kidney. Development 131: 4085-4093
72. Takei K, Mignery GA, Mugnaini E, Sudhof TC, De Camilli P (1994) Inositol 1,4,5-trisphosphate receptor causes formation of ER cisternal stacks in transfected fibroblasts and in cerebellar Purkinje cells. Neuron 12: 327-342
73. Tsiokas L, Arnould T, Zhu C, Kim E, Walz G, Sukhatme VP (1999) Specific association of the gene product of PKD2 with the TRPC1 channel. Proc Natl Acad Sci USA 96: 3934-3939
74. Tsiokas L, Kim E, Arnould T, Sukhatme VP, Walz G (1997) Homo-and heterodimeric interactions between the gene products of PKD1 and PKD2. Proc Natl Acad Sci USA 94: 6965-6970
75. Tsuruda PR, Julius D, Minor Jr DL (2006) Coiled coils direct assembly of a cold-activated TRP channel. Neuron 51: 201-212
76. Wessely O, Obara T (2008) Fish and frogs: models for vertebrate cilia signaling. Front Biosci 13: 1866-1880
77. Wiener R, Haitin Y, Shamgar L, Fernández-Alonso MC, Martos A, Chomsky-Hecht O, Rivas G, Attali B, Hirsch JA (2008) The KCNQ1 (Kv7.1) COOH terminus, a multitiered scaffold for subunit assembly and protein interaction. J Biol Chem 283: 5815-5830
78. Wright R, Keller G, Gould SJ, Subramani S, Rine J (1990) Cell-type control of membrane biogenesis induced by HMG-CoA reductase. New Biol 2: 915-921
79. Wu G, Somlo S (2000) Molecular genetics and mechanism of autosomal dominant polycystic kidney disease. Mol Genet Metab 69: 1-15 (Pubitemid 30097298)
80. Wu Y, Dai XQ, Li Q, Chen CX, Mai W, Hussain Z, Long W, Montalbetti N, Li G, Glynne R, Wang S, Cantiello HF, Wu G, Chen XZ (2006) Kinesin-2 mediates physical and functional interactions between polycystin-2 and fibrocystin. Hum Mol Genet 15: 3280-3292
81. Xu Q, Minor Jr DL (2009) Crystal structure of a trimeric form of the K(V)7.1 (KCNQ1) a-domain tail coiled-coil reveals structural plasticity and context dependent changes in a putative coiled-coil trimerization motif. Protein Sci 18: 2100-2114, PubMed PMID: 19693805
82. Yoder BK, Hou X, Guay-Woodford LM (2002) The polycystic kidney disease proteins, polycystin-1, polycystin-2, polaris, and cystin, are co-localized in renal cilia. J Am Soc Nephrol 13: 2508-2516
83. Yu Y, Ulbrich MH, Li MH, Buraei Z, Chen XZ, Ong AC, Tong L, Isacoff EY, Yang J (2009) Structural and molecular basis of the assembly of the TRPP2/PKD1 complex. Proc Natl Acad Sci USA 106: 11558-11563
84. Zhang P, Luo Y, Chasan B, Gonzalez-Perrett S, Montalbetti N, Timpanaro GA, Cantero Mdel R, Ramos AJ, Goldmann WH, Zhou J, Cantiello HF (2009) The multimeric structure of poly-cystin-2 (TRPP2): structural-functional correlates of homo-and hetero-multimers with TRPC1. Hum Mol Genet 18: 1238-1251