Exome capture reveals ZNF423 and CEP164 mutations, linking renal ciliopathies to DNA damage response signaling

Cell

Volumn 150, Issue 3, 2012, Pages 533-548

  Chaki, Moumita ^a   Airik, Rannar ^a   Ghosh, Amiya K ^a   Giles, Rachel H ^c   Chen, Rui ^d   Slaats, Gisela G ^c   Wang, Hui ^d   Hurd, Toby W ^a   Zhou, Weibin ^a   Cluckey, Andrew ^a   Gee, Heon Yung ^a   Ramaswami, Gokul ^a   Hong, Chen Jei ^e   Hamilton, Bruce A ^e   Červenka, Igor ^f   Ganji, Ranjani Sri ^f   Bryja, Vitezslav ^f,g   Arts, Heleen H ^h   Van Reeuwijk, Jeroen ^h   Oud, MacHteld M ^h   Letteboer, Stef J F ^h   Roepman, Ronald ^h   Husson, Hervé ⁱ   Ibraghimov Beskrovnaya, Oxana ⁱ   Yasunaga, Takayuki ^j   Walz, Gerd ^j   Eley, Lorraine ^k   Sayer, John A ^k   Schermer, Bernhard ^l   Liebau, Max C ^l   Benzing, Thomas ^l   Le Corre, Stephanie ^o   Drummond, Iain ^o   Janssen, Sabine ^a   Allen, Susan J ^a   Natarajan, Sivakumar ^a   O'Toole, John F ^p   Attanasio, Massimo ^q   Saunier, Sophie ^r   Antignac, Corinne ^r   Koenekoop, Robert K ^s   Ren, Huanan ^s   Lopez, Irma ^s   Nayir, Ahmet ^t   Stoetzel, Corinne ^u   Dollfus, Helene ^u   Massoudi, Rustin ^v   Gleeson, Joseph G ^v   Andreoli, Sharon P ^w   Doherty, Dan G ^x   Lindstrad, Anna ^y   Golzio, Christelle ^y   Katsanis, Nicholas ^y   Pape, Lars ^z   Abboud, Emad B ^aa   Al Rajhi, Ali A ^aa   Lewis, Richard A ^d   Omran, Heymut ^ab   Lee, Eva Y H P ^ac   Wang, Shaohui ^ac   Sekiguchi, Joann M ^b   Saunders, Rudel ^b   Johnson, Colin A ^ad   Garner, Elizabeth ^ae   Vanselow, Katja ^af   Andersen, Jens S ^af   Shlomai, Joseph ⁿ   Nurnberg, Gudrun ^m   Nurnberg, Peter ^m   Levy, Shawn ^ag   Smogorzewska, Agata ^ae   Otto, Edgar A ^a   Hildebrandt, Friedhelm ^a,b,ah   more..

^a UNIVERSITY OF MICHIGAN   (United States)

^b UNIVERSITY OF MICHIGAN MEDICAL SCHOOL   (United States)

^c UNIVERSITY MEDICAL CENTER UTRECHT   (Netherlands)

^d BAYLOR COLLEGE OF MEDICINE   (United States)

^e UNIVERSITY OF CALIFORNIA   (United States)

^f MASARYK UNIVERSITY   (Czech Republic)

^g INSTITUTE OF MATHEMATICS   (Czech Republic)

^h RADBOUD UNIVERSITY MEDICAL CENTER   (Netherlands)

ⁱ GENZYME CORPORATION   (United States)

^j UNIVERSITY OF FREIBURG   (Germany)

^k NEWCASTLE UNIVERSITY   (United Kingdom)

^l UNIVERSITY OF COLOGNE   (Germany)

^m UNIVERSITY OF COLOGNE   (Germany)

ⁿ HEBREW UNIVERSITY   (Israel)

^o HARVARD MEDICAL SCHOOL   (United States)

^p METROHEALTH MEDICAL CENTER   (United States)

^q UNIVERSITY OF TEXAS SOUTHWESTERN MEDICAL CENTER   (United States)

^r INSERM   (France)

^s MONTREAL CHILDREN S HOSPITAL   (Canada)

^t ISTANBUL UNIVERSITY   (Turkey)

^u UNIVERSITÉ DE STRASBOURG   (France)

^v UNIVERSITY OF CALIFORNIA SAN DIEGO   (United States)

^w RILEY HOSPITAL FOR CHILDREN   (United States)

^x UNIVERSITY OF WASHINGTON   (United States)

^y DUKE UNIVERSITY MEDICAL CENTER   (United States)

^z HANNOVER MEDICAL SCHOOL   (Germany)

^aa KING KHALED EYE SPECIALIST HOSPITAL   (Saudi Arabia)

^ab UNIVERSITY HOSPITAL MÜNSTER   (Germany)

^ac UNIVERSITY OF CALIFORNIA   (United States)

^ad ST JAMES S UNIVERSITY HOSPITAL   (United Kingdom)

^ae ROCKEFELLER UNIVERSITY   (United States)

^af UNIVERSITY OF SOUTHERN DENMARK   (Denmark)

^ag HUDSONALPHA INSTITUTE FOR BIOTECHNOLOGY   (United States)

^ah HOWARD HUGHES MEDICAL INSTITUTE   (United States)

more..

Author keywords

[No Author keywords available]

Indexed keywords

ANIMAL TISSUE; ARTICLE; CEP164 GENE; CONTROLLED STUDY; DEGENERATIVE DISEASE; DNA DAMAGE; EXOME; GENE; GENE MUTATION; GENE SEQUENCE; GENE SILENCING; GENETIC ASSOCIATION; HUMAN; HUMAN CELL; MRE11 GENE; NEPHRONOPHTHISIS RELATED CILIOPATHIES; NONHUMAN; PHENOTYPE; PRIORITY JOURNAL; SIGNAL TRANSDUCTION; ZEBRA FISH; ZNF423 GENE;

DANIO RERIO;

EID: 84864584531     PISSN: 00928674     EISSN: 10974172     Source Type: Journal    
DOI: 10.1016/j.cell.2012.06.028     Document Type: Article

References (67)

1. W.A. Alcaraz, D.A. Gold, E. Raponi, P.M. Gent, D. Concepcion, and B.A. Hamilton Zfp423 controls proliferation and differentiation of neural precursors in cerebellar vermis formation Proc. Natl. Acad. Sci. USA 103 2006 19424 19429
2. W.A. Alcaraz, E. Chen, P. Valdes, E. Kim, Y.H. Lo, J. Vo, and B.A. Hamilton Modifier genes and non-genetic factors reshape anatomical deficits in Zfp423-deficient mice Hum. Mol. Genet. 20 2011 3822 3830
3. S.J. Ansley, J.L. Badano, O.E. Blacque, J. Hill, B.E. Hoskins, C.C. Leitch, J.C. Kim, A.J. Ross, E.R. Eichers, and T.M. Teslovich Basal body dysfunction is a likely cause of pleiotropic Bardet-Biedl syndrome Nature 425 2003 628 633
4. M. Attanasio, N.H. Uhlenhaut, V.H. Sousa, J.F. O'Toole, E. Otto, K. Anlag, C. Klugmann, A.C. Treier, J. Helou, and J.A. Sayer Loss of GLIS2 causes nephronophthisis in humans and mice by increased apoptosis and fibrosis Nat. Genet. 39 2007 1018 1024
5. S. Blachon, J. Gopalakrishnan, Y. Omori, A. Polyanovsky, A. Church, D. Nicastro, J. Malicki, and T. Avidor-Reiss Drosophila asterless and vertebrate Cep152 Are orthologs essential for centriole duplication Genetics 180 2008 2081 2094
6. V. Bryja, G. Schulte, N. Rawal, A. Grahn, and E. Arenas Wnt-5a induces Dishevelled phosphorylation and dopaminergic differentiation via a CK1-dependent mechanism J. Cell Sci. 120 2007 586 595
7. N.O. Bukanov, L.A. Smith, K.W. Klinger, S.R. Ledbetter, and O. Ibraghimov-Beskrovnaya Long-lasting arrest of murine polycystic kidney disease with CDK inhibitor roscovitine Nature 444 2006 949 952
8. M. Chaki, J. Hoefele, S.J. Allen, G. Ramaswami, S. Janssen, C. Bergmann, J.R. Heckenlively, E.A. Otto, and F. Hildebrandt Genotype-phenotype correlation in 440 patients with NPHP-related ciliopathies Kidney Int. 80 2011 1239 1245
9. L.E. Cheng, J. Zhang, and R.R. Reed The transcription factor Zfp423/OAZ is required for cerebellar development and CNS midline patterning Dev. Biol. 307 2007 43 52
10. A. Ciccia, and S.J. Elledge The DNA damage response: making it safe to play with knives Mol. Cell 40 2010 179 204
11. S.J. Doxsey, P. Stein, L. Evans, P.D. Calarco, and M. Kirschner Pericentrin, a highly conserved centrosome protein involved in microtubule organization Cell 76 1994 639 650
12. V. Edmond, E. Moysan, S. Khochbin, P. Matthias, C. Brambilla, E. Brambilla, S. Gazzeri, and B. Eymin Acetylation and phosphorylation of SRSF2 control cell fate decision in response to cisplatin EMBO J. 30 2011 510 523
13. E. Elias, N. Lalun, M. Lorenzato, L. Blache, P. Chelidze, M.F. O'Donohue, D. Ploton, and H. Bobichon Cell-cycle-dependent three-dimensional redistribution of nuclear proteins, P 120, pKi-67, and SC 35 splicing factor, in the presence of the topoisomerase I inhibitor camptothecin Exp. Cell Res. 291 2003 176 188
14. E. Fischer, E. Legue, A. Doyen, F. Nato, J.F. Nicolas, V. Torres, M. Yaniv, and M. Pontoglio Defective planar cell polarity in polycystic kidney disease Nat. Genet. 38 2006 21 23
15. G.G. Germino Linking cilia to Wnts Nat. Genet. 37 2005 455 457
16. S. Graser, Y.D. Stierhof, S.B. Lavoie, O.S. Gassner, S. Lamla, M. Le Clech, and E.A. Nigg Cep164, a novel centriole appendage protein required for primary cilium formation J. Cell Biol. 179 2007 321 330
17. E. Griffith, S. Walker, C.A. Martin, P. Vagnarelli, T. Stiff, B. Vernay, N. Al Sanna, A. Saggar, B. Hamel, and W.C. Earnshaw Mutations in pericentrin cause Seckel syndrome with defective ATR-dependent DNA damage signaling Nat. Genet. 40 2008 232 236
18. D.F. Gudbjartsson, K. Jonasson, M.L. Frigge, and A. Kong Allegro, a new computer program for multipoint linkage analysis Nat. Genet. 25 2000 12 13
19. F. Hildebrandt, S.F. Heeringa, F. Rüschendorf, M. Attanasio, G. Nürnberg, C. Becker, D. Seelow, N. Huebner, G. Chernin, and C.N. Vlangos A systematic approach to mapping recessive disease genes in individuals from outbred populations PLoS Genet. 5 2009 e1000353
20. F. Hildebrandt, T. Benzing, and N. Katsanis Ciliopathies N. Engl. J. Med. 364 2011 1533 1543
21. D. Huangfu, and K.V. Anderson Cilia and Hedgehog responsiveness in the mouse Proc. Natl. Acad. Sci. USA 102 2005 11325 11330
22. D. Huangfu, A. Liu, A.S. Rakeman, N.S. Murcia, L. Niswander, and K.V. Anderson Hedgehog signalling in the mouse requires intraflagellar transport proteins Nature 426 2003 83 87
23. L. Janderová-Rossmeislová, Z. Nováková, J. Vlasáková, V. Philimonenko, P. Hozák, and Z. Hodný PML protein association with specific nucleolar structures differs in normal, tumor and senescent human cells J. Struct. Biol. 159 2007 56 70
24. E. Kalay, G. Yigit, Y. Aslan, K.E. Brown, E. Pohl, L.S. Bicknell, H. Kayserili, Y. Li, B. Tüysüz, and G. Nürnberg CEP152 is a genome maintenance protein disrupted in Seckel syndrome Nat. Genet. 43 2011 23 26
25. L. Kruglyak, M.J. Daly, M.P. Reeve-Daly, and E.S. Lander Parametric and nonparametric linkage analysis: a unified multipoint approach Am. J. Hum. Genet. 58 1996 1347 1363
26. M.C. Ku, S. Stewart, and A. Hata Poly(ADP-ribose) polymerase 1 interacts with OAZ and regulates BMP-target genes Biochem. Biophys. Res. Commun. 311 2003 702 707
27. Q. Liu, G. Tan, N. Levenkova, T. Li, E.N. Pugh Jr., J.J. Rux, D.W. Speicher, and E.A. Pierce The proteome of the mouse photoreceptor sensory cilium complex Mol. Cell. Proteomics 6 2007 1299 1317
28. S.L. Maude, and G.H. Enders Cdk inhibition in human cells compromises chk1 function and activates a DNA damage response Cancer Res. 65 2005 780 786
29. M. Murga, S. Bunting, M.F. Montaña, R. Soria, F. Mulero, M. Cañamero, Y. Lee, P.J. McKinnon, A. Nussenzweig, and O. Fernandez-Capetillo A mouse model of ATR-Seckel shows embryonic replicative stress and accelerated aging Nat. Genet. 41 2009 891 898
30. S.B. Ng, E.H. Turner, P.D. Robertson, S.D. Flygare, A.W. Bigham, C. Lee, T. Shaffer, M. Wong, A. Bhattacharjee, and E.E. Eichler Targeted capture and massively parallel sequencing of 12 human exomes Nature 461 2009 272 276
31. M. O'Driscoll, V.L. Ruiz-Perez, C.G. Woods, P.A. Jeggo, and J.A. Goodship A splicing mutation affecting expression of ataxia-telangiectasia and Rad3-related protein (ATR) results in Seckel syndrome Nat. Genet. 33 2003 497 501
32. E.A. Otto, T.W. Hurd, R. Airik, M. Chaki, W. Zhou, C. Stoetzel, S.B. Patil, S. Levy, A.K. Ghosh, and C.A. Murga-Zamalloa Candidate exome capture identifies mutation of SDCCAG8 as the cause of a retinal-renal ciliopathy Nat. Genet. 42 2010 840 850
33. E.A. Otto, G. Ramaswami, S. Janssen, M. Chaki, S.J. Allen, W. Zhou, R. Airik, T.W. Hurd, A.K. Ghosh, M.T. Wolf GPN Study Group Mutation analysis of 18 nephronophthisis associated ciliopathy disease genes using a DNA pooling and next generation sequencing strategy J. Med. Genet. 48 2011 105 116
34. Y.R. Pan, and E.Y. Lee UV-dependent interaction between Cep164 and XPA mediates localization of Cep164 at sites of DNA damage and UV sensitivity Cell Cycle 8 2009 655 664
35. K. Piontek, L.F. Menezes, M.A. Garcia-Gonzalez, D.L. Huso, and G.G. Germino A critical developmental switch defines the kinetics of kidney cyst formation after loss of Pkd1 Nat. Med. 13 2007 1490 1495
36. A. Rauch, C.T. Thiel, D. Schindler, U. Wick, Y.J. Crow, A.B. Ekici, A.J. van Essen, T.O. Goecke, L. Al-Gazali, and K.H. Chrzanowska Mutations in the pericentrin (PCNT) gene cause primordial dwarfism Science 319 2008 816 819
37. H.C. Reinhardt, I.G. Cannell, S. Morandell, and M.B. Yaffe Is post-transcriptional stabilization, splicing and translation of selective mRNAs a key to the DNA damage response? Cell Cycle 10 2011 23 27
38. M.E. Robu, J.D. Larson, A. Nasevicius, S. Beiraghi, C. Brenner, S.A. Farber, and S.C. Ekker p53 activation by knockdown technologies PLoS Genet. 3 2007 e78
39. L. Sang, J.J. Miller, K.C. Corbit, R.H. Giles, M.J. Brauer, E.A. Otto, L.M. Baye, X. Wen, S.J. Scales, and M. Kwong Mapping the NPHP-JBTS-MKS protein network reveals ciliopathy disease genes and pathways Cell 145 2011 513 528
40. K. Savitsky, A. Bar-Shira, S. Gilad, G. Rotman, Y. Ziv, L. Vanagaite, D.A. Tagle, S. Smith, T. Uziel, and S. Sfez A single ataxia telangiectasia gene with a product similar to PI-3 kinase Science 268 1995 1749 1753
41. J.A. Sayer, E.A. Otto, J.F. O'Toole, G. Nurnberg, M.A. Kennedy, C. Becker, H.C. Hennies, J. Helou, M. Attanasio, and B.V. Fausett The centrosomal protein nephrocystin-6 is mutated in Joubert syndrome and activates transcription factor ATF4 Nat. Genet. 38 2006 674 681
42. T. Schäfer, M. Pütz, S. Lienkamp, A. Ganner, A. Bergbreiter, H. Ramachandran, V. Gieloff, M. Gerner, C. Mattonet, and P.G. Czarnecki Genetic and physical interaction between the NPHP5 and NPHP6 gene products Hum. Mol. Genet. 17 2008 3655 3662
43. M. Simons, J. Gloy, A. Ganner, A. Bullerkotte, M. Bashkurov, C. Krönig, B. Schermer, T. Benzing, O.A. Cabello, and A. Jenny Inversin, the gene product mutated in nephronophthisis type II, functions as a molecular switch between Wnt signaling pathways Nat. Genet. 37 2005 537 543
44. S. Sivasubramaniam, X. Sun, Y.R. Pan, S. Wang, and E.Y. Lee Cep164 is a mediator protein required for the maintenance of genomic stability through modulation of MDC1, RPA, and CHK1 Genes Dev. 22 2008 587 600
45. A. Smogorzewska, S. Matsuoka, P. Vinciguerra, E.R. McDonald 3rd, K.E. Hurov, J. Luo, B.A. Ballif, S.P. Gygi, K. Hofmann, A.D. D'Andrea, and S.J. Elledge Identification of the FANCI protein, a monoubiquitinated FANCD2 paralog required for DNA repair Cell 129 2007 289 301
46. G.S. Stewart, R.S. Maser, T. Stankovic, D.A. Bressan, M.I. Kaplan, N.G. Jaspers, A. Raams, P.J. Byrd, J.H. Petrini, and A.M. Taylor The DNA double-strand break repair gene hMRE11 is mutated in individuals with an ataxia-telangiectasia-like disorder Cell 99 1999 577 587
47. K. Strauch, R. Fimmers, T. Kurz, K.A. Deichmann, T.F. Wienker, and M.P. Baur Parametric and nonparametric multipoint linkage analysis with imprinting and two-locus-trait models: application to mite sensitization Am. J. Hum. Genet. 66 2000 1945 1957
48. I. Szczerbal, and J.M. Bridger Association of adipogenic genes with SC-35 domains during porcine adipogenesis Chromosome Res. 18 2010 887 895
49. E.M. Valente, J.L. Silhavy, F. Brancati, G. Barrano, S.R. Krishnaswami, M. Castori, M.A. Lancaster, E. Boltshauser, L. Boccone, L. Al-Gazali International Joubert Syndrome Related Disorders Study Group Mutations in CEP290, which encodes a centrosomal protein, cause pleiotropic forms of Joubert syndrome Nat. Genet. 38 2006 623 625
50. R. Xiao, Y. Sun, J.H. Ding, S. Lin, D.W. Rose, M.G. Rosenfeld, X.D. Fu, and X. Li Splicing regulator SC35 is essential for genomic stability and cell proliferation during mammalian organogenesis Mol. Cell. Biol. 27 2007 5393 5402
51. J. Yu, T.J. Carroll, J. Rajagopal, A. Kobayashi, Q. Ren, and A.P. McMahon A Wnt7b-dependent pathway regulates the orientation of epithelial cell division and establishes the cortico-medullary axis of the mammalian kidney Development 136 2009 161 171
52. Bentley, D.R., Balasubramanian, S., Swerdlow, H.P., Smith, G.P., Milton, J., Brown, C.G., Hall, K.P., Evers, D.J., Barnes, C.L., Bignell, H.R., et al. (2008). Accurate whole human genome sequencing using reversible terminator chemistry. Nature 456, 53-59.
53. Bryja, V., Gradl, D., Schambony, A., Arenas, E., and Schulte, G. (2007a). Beta-arrestin is a necessary component of Wnt/beta-catenin signaling in vitro and in vivo. Proc. Natl. Acad. Sci. USA 104, 6690-6695.
54. Bryja, V., Schulte, G., Rawal, N., Grahn, A., and Arenas, E. (2007b). Wnt-5a induces Dishevelled phosphorylation and dopaminergic differentiation via a CK1-dependent mechanism. J. Cell Sci. 120, 586-595.
55. Bukanov, N.O., Smith, L.A., Klinger, K.W., Ledbetter, S.R., and Ibraghimov-Beskrovnaya, O. (2006). Long-lasting arrest of murine polycystic kidney disease with CDK inhibitor roscovitine. Nature 444, 949-952.
56. Coene, K.L., Mans, D.A., Boldt, K., Gloeckner, C.J., van Reeuwijk, J., Bolat, E., Roosing, S., Letteboer, S.J., Peters, T.A., Cremers, F.P., et al. (2011). The ciliopathy-associated protein homologs RPGRIP1 and RPGRIP1L are linked to cilium integrity through interaction with Nek4 serine/threonine kinase. Hum. Mol. Genet. 20, 3592-3605.
57. Giorgio, G., Alfieri, M., Prattichizzo, C., Zullo, A., Cairo, S., and Franco, B. (2007). Functional characterization of the OFD1 protein reveals a nuclear localization and physical interaction with subunits of a chromatin remodeling complex. Mol. Biol. Cell 18, 4397-4404.
58. Habbig, S., Bartram, M.P., Müller, R.U., Schwarz, R., Andriopoulos, N., Chen, S., Sägmüller, J.G., Hoehne, M., Burst, V., Liebau, M.C., et al. (2011). NPHP4, a cilia-associated protein, negatively regulates the Hippo pathway. J. Cell Biol. 193, 633-642.
59. Letteboer, S.J., and Roepman, R. (2008). Versatile screening for binary protein-protein interactions by yeast two-hybrid mating. Methods Mol. Biol. 484, 145-159.
60. Liebau, M.C., Höpker, K., Müller, R.U., Schmedding, I., Zank, S., Schairer, B., Fabretti, F., Höhne, M., Bartram, M.P., Dafinger, C., et al. (2011). Nephrocystin-4 regulates Pyk2-induced tyrosine phosphorylation of nephrocystin-1 to control targeting to monocilia. J. Biol. Chem. 286, 14237-14245.
61. Otto, E.A., Schermer, B., Obara, T., O'Toole, J.F., Hiller, K.S., Mueller, A.M., Ruf, R.G., Hoefele, J., Beekmann, F., Landau, D., et al. (2003). Mutations in INVS encoding inversin cause nephronophthisis type 2, linking renal cystic disease to the function of primary cilia and left-right axis determination. Nat. Genet. 34, 413-420.
62. Otto, E.A., Ramaswami, G., Janssen, S., Chaki, M., Allen, S.J., Zhou, W., Airik, R., Hurd, T.W., Ghosh, A.K., Wolf, M.T., et al.; GPN Study Group (2011). Mutation analysis of 18 nephronophthisis associated ciliopathy disease genes using a DNA pooling and next generation sequencing strategy. J. Med. Genet. 48, 105-116.
63. Sayer, J.A., Otto, E.A., O'Toole, J.F., Nurnberg, G., Kennedy, M.A., Becker, C., Hennies, H.C., Helou, J., Attanasio, M., Fausett, B.V., et al. (2006). The centrosomal protein nephrocystin-6 is mutated in Joubert syndrome and activates transcription factor ATF4. Nat. Genet. 38, 674-681.
64. Sivasubramaniam, S., Sun, X., Pan, Y.R., Wang, S., and Lee, E.Y. (2008). Cep164 is a mediator protein required for the maintenance of genomic stability through modulation of MDC1, RPA, and CHK1. Genes Dev. 22, 587-600.
65. Smith, L.A., Bukanov, N.O., Husson, H., Russo, R.J., Barry, T.C., Taylor, A.L., Beier, D.R., and Ibraghimov-Beskrovnaya, O. (2006). Development of polycystic kidney disease in juvenile cystic kidney mice: insights into pathogenesis, ciliary abnormalities, and common features with human disease. J. Am. Soc. Nephrol. 17, 2821-2831.
66. Smogorzewska, A., Matsuoka, S., Vinciguerra, P., McDonald, E.R., 3rd, Hurov, K.E., Luo, J., Ballif, B.A., Gygi, S.P., Hofmann, K., D'Andrea, A.D., and Elledge, S.J. (2007). Identification of the FANCI protein, a monoubiquitinated FANCD2 paralog required for DNA repair. Cell 129, 289-301.
67. Zhou, W., Dai, J., Attanasio, M., and Hildebrandt, F. (2010). Nephrocystin-3 is required for ciliary function in zebrafish embryos. Am. J. Physiol. Renal Physiol. 299, F55-F62.