polycystic kidney diseases

Summary

Summary: Hereditary diseases that are characterized by the progressive expansion of a large number of tightly packed CYSTS within the KIDNEYS. They include diseases with autosomal dominant and autosomal recessive inheritance.

Top Publications

  1. Park E, Woo Y, Park J. Polycystic kidney disease and therapeutic approaches. BMB Rep. 2011;44:359-68 pubmed publisher
    ..In this review, we will discuss recent approaches to PKD therapy. It provides important information regarding potential targets for PKD...
  2. Winyard P, Jenkins D. Putative roles of cilia in polycystic kidney disease. Biochim Biophys Acta. 2011;1812:1256-62 pubmed publisher
    ..Understanding how cilia fit into the other aspects of polycystic kidney disease biology is the challenge for the next decade. This article is part of a Special Issue entitled: Polycystic Kidney Disease...
  3. Gao J, Zhou H, Lei T, Zhou L, Li W, Li X, et al. Curcumin inhibits renal cyst formation and enlargement in vitro by regulating intracellular signaling pathways. Eur J Pharmacol. 2011;654:92-9 pubmed publisher
    ..These results define that curcumin inhibits renal cyst formation and enlargement and suggest that curcumin might be developed as a candidate drug for polycystic kidney disease...
  4. Togawa H, Nakanishi K, Mukaiyama H, Hama T, Shima Y, Sako M, et al. Epithelial-to-mesenchymal transition in cyst lining epithelial cells in an orthologous PCK rat model of autosomal-recessive polycystic kidney disease. Am J Physiol Renal Physiol. 2011;300:F511-20 pubmed publisher
    ..Our study clarified the nephron segment-specific cyst profile related to EMT in PCK rats. EMT may play a key role in polycystic kidney disease...
  5. Shalom O, Shalva N, Altschuler Y, Motro B. The mammalian Nek1 kinase is involved in primary cilium formation. FEBS Lett. 2008;582:1465-70 pubmed publisher
    Recent studies implicate primary cilium (PC) proteins in the etiologies of various polycystic kidney diseases (PKD). NIMA-related kinases (NRKs) are conserved serine/threonine kinases, which are usually defined as 'mitotic kinases'...
  6. Town T, Breunig J, Sarkisian M, Spilianakis C, Ayoub A, Liu X, et al. The stumpy gene is required for mammalian ciliogenesis. Proc Natl Acad Sci U S A. 2008;105:2853-8 pubmed publisher
    ..Therefore, stumpy is essential for ciliogenesis and may be involved in the pathogenesis of human congenital malformations such as HC and PKD...
  7. Patel V, Chowdhury R, Igarashi P. Advances in the pathogenesis and treatment of polycystic kidney disease. Curr Opin Nephrol Hypertens. 2009;18:99-106 pubmed publisher
    ..Mouse models of PKD, especially those with mutations in genes that are orthologous to human disease genes, have provided insights into the pathogenesis of cyst formation and advanced the preclinical testing of new drugs...
  8. Li H, Sheppard D. Therapeutic potential of cystic fibrosis transmembrane conductance regulator (CFTR) inhibitors in polycystic kidney disease. BioDrugs. 2009;23:203-16 pubmed publisher
    ..The demonstration that CFTR inhibitors retard cyst expansion and kidney enlargement in mouse models of ADPKD provides proof of concept for the use of small-molecule CFTR inhibitors in the treatment of ADPKD...
  9. Verdeguer F, Le Corre S, Fischer E, Callens C, Garbay S, Doyen A, et al. A mitotic transcriptional switch in polycystic kidney disease. Nat Med. 2010;16:106-10 pubmed publisher
    ..This association suggests that HNF-1beta is a bookmarking factor that is necessary for reopening the chromatin of target genes after mitotic silencing...

More Information

Publications73

  1. Novalic Z, van der Wal A, Leonhard W, Koehl G, Breuning M, Geissler E, et al. Dose-dependent effects of sirolimus on mTOR signaling and polycystic kidney disease. J Am Soc Nephrol. 2012;23:842-53 pubmed publisher
    ..Mechanisms to increase bioavailability or to target mTOR inhibitors more specifically to kidneys, alone or in combination with other compounds, may improve the potential for these therapies in PKD...
  2. Williams C, Li C, Kida K, Inglis P, Mohan S, Semenec L, et al. MKS and NPHP modules cooperate to establish basal body/transition zone membrane associations and ciliary gate function during ciliogenesis. J Cell Biol. 2011;192:1023-41 pubmed publisher
  3. Fedeles S, Tian X, Gallagher A, Mitobe M, Nishio S, Lee S, et al. A genetic interaction network of five genes for human polycystic kidney and liver diseases defines polycystin-1 as the central determinant of cyst formation. Nat Genet. 2011;43:639-47 pubmed publisher
  4. Pandey P, Qin S, Ho J, Zhou J, Kreidberg J. Systems biology approach to identify transcriptome reprogramming and candidate microRNA targets during the progression of polycystic kidney disease. BMC Syst Biol. 2011;5:56 pubmed publisher
    ..miRNAs are small, regulatory non-coding RNAs, implicated in a wide spectrum of biological processes. Their expression levels are altered in several diseases including kidney cancer, diabetic nephropathy and PKD...
  5. Dai B, Liu Y, Mei C, Fu L, Xiong X, Zhang Y, et al. Rosiglitazone attenuates development of polycystic kidney disease and prolongs survival in Han:SPRD rats. Clin Sci (Lond). 2010;119:323-33 pubmed publisher
  6. Tran U, Zakin L, Schweickert A, Agrawal R, D ger R, Blum M, et al. The RNA-binding protein bicaudal C regulates polycystin 2 in the kidney by antagonizing miR-17 activity. Development. 2010;137:1107-16 pubmed publisher
    ..Based on these data, we propose that the kidney phenotype in Bicc1(-/-) mutant mice is caused by dysregulation of a microRNA-based translational control mechanism...
  7. Collin G, Won J, Hicks W, Cook S, Nishina P, Naggert J. Meckelin is necessary for photoreceptor intraciliary transport and outer segment morphogenesis. Invest Ophthalmol Vis Sci. 2012;53:967-74 pubmed publisher
    ..In this study, the retinal morphology and ciliary function in a mouse model for Meckel Syndrome Type 3 (MKS3) throughout the course of photoreceptor development was examined...
  8. Patel V, Li L, Cobo Stark P, Shao X, Somlo S, Lin F, et al. Acute kidney injury and aberrant planar cell polarity induce cyst formation in mice lacking renal cilia. Hum Mol Genet. 2008;17:1578-90 pubmed publisher
    ..We conclude that primary cilia are required for the maintenance of planar cell polarity in the mammalian kidney and that acute kidney injury exacerbates cystic disease...
  9. Okada S, Misaka T, Tanaka Y, Matsumoto I, Ishibashi K, Sasaki S, et al. Aquaporin-11 knockout mice and polycystic kidney disease animals share a common mechanism of cyst formation. FASEB J. 2008;22:3672-84 pubmed publisher
    ..We also demonstrated the activation of apoptosis via the ER-stress pathway in the proximal tubule cells with vacuolized ER. These results provide new insights into the physiological roles of AQP11...
  10. Luyten A, Su X, Gondela S, Chen Y, Rompani S, Takakura A, et al. Aberrant regulation of planar cell polarity in polycystic kidney disease. J Am Soc Nephrol. 2010;21:1521-32 pubmed publisher
    ..Taken together, our data suggest that PC1 controls oriented cell division and that aberrant PCP signaling contributes to cystogenesis...
  11. Harris P, Torres V. Polycystic kidney disease. Annu Rev Med. 2009;60:321-37 pubmed publisher
    ..Rare, syndromic forms of PKD also include defects of the eye, central nervous system, digits, and/or neural tube and highlight the role of cilia and pathways such as Wnt and Hh in their pathogenesis...
  12. Renken C, Fischer D, Kundt G, Gretz N, Haffner D. Inhibition of mTOR with sirolimus does not attenuate progression of liver and kidney disease in PCK rats. Nephrol Dial Transplant. 2011;26:92-100 pubmed publisher
    ..We examined the effects of sirolimus in PCK rats, an orthologous animal model of human ARPKD...
  13. Belibi F, Zafar I, Ravichandran K, Segvic A, Jani A, Ljubanovic D, et al. Hypoxia-inducible factor-1? (HIF-1?) and autophagy in polycystic kidney disease (PKD). Am J Physiol Renal Physiol. 2011;300:F1235-43 pubmed publisher
    ..The first demonstration of autophagosomes in PKD kidneys is reported. Inhibition of HIF-1? did not have a therapeutic effect...
  14. Sang L, Miller J, Corbit K, Giles R, Brauer M, Otto E, et al. Mapping the NPHP-JBTS-MKS protein network reveals ciliopathy disease genes and pathways. Cell. 2011;145:513-28 pubmed publisher
    ..Our study further illustrates the power of linking proteomic networks and human genetics to uncover critical disease pathways. ..
  15. Dweep H, Sticht C, Kharkar A, Pandey P, Gretz N. Parallel analysis of mRNA and microRNA microarray profiles to explore functional regulatory patterns in polycystic kidney disease: using PKD/Mhm rat model. PLoS ONE. 2013;8:e53780 pubmed publisher
    ..We further describe novel miRNAs and their possible targets in ADPKD, which will open new avenues to understand the pathogenesis of human ADPKD. Furthermore they could serve as a useful resource for anti-fibrotic therapeutics...
  16. Gattone V, Chen N, Sinders R, Seifert M, Duan D, Martin D, et al. Calcimimetic inhibits late-stage cyst growth in ADPKD. J Am Soc Nephrol. 2009;20:1527-32 pubmed publisher
    ..The benefit of R-568 alone suggests calcium-sensing receptor modulation may have additional inhibitory effects on late-stage cyst growth resulting from a direct modulation of intracellular calcium...
  17. Tallila J, Jakkula E, Peltonen L, Salonen R, Kestila M. Identification of CC2D2A as a Meckel syndrome gene adds an important piece to the ciliopathy puzzle. Am J Hum Genet. 2008;82:1361-7 pubmed publisher
  18. Garcia Gonzalo F, Corbit K, Sirerol Piquer M, Ramaswami G, Otto E, Noriega T, et al. A transition zone complex regulates mammalian ciliogenesis and ciliary membrane composition. Nat Genet. 2011;43:776-84 pubmed publisher
    ..Thus, a transition zone complex of Meckel and Joubert syndrome proteins regulates ciliary assembly and trafficking, suggesting that transition zone dysfunction is the cause of these ciliopathies. ..
  19. Lehman J, Michaud E, Schoeb T, Aydin Son Y, Miller M, Yoder B. The Oak Ridge Polycystic Kidney mouse: modeling ciliopathies of mice and men. Dev Dyn. 2008;237:1960-71 pubmed publisher
  20. Natoli T, Smith L, Rogers K, Wang B, Komarnitsky S, Budman Y, et al. Inhibition of glucosylceramide accumulation results in effective blockade of polycystic kidney disease in mouse models. Nat Med. 2010;16:788-92 pubmed publisher
    ..Taken together, our data suggest that inhibition of GlcCer synthesis represents a new and effective treatment option for PKD...
  21. Olsan E, Mukherjee S, Wulkersdorfer B, Shillingford J, Giovannone A, Todorov G, et al. Signal transducer and activator of transcription-6 (STAT6) inhibition suppresses renal cyst growth in polycystic kidney disease. Proc Natl Acad Sci U S A. 2011;108:18067-72 pubmed publisher
    ..Treatment of PKD mice with this drug leads to amelioration of the renal cystic disease similar to genetic STAT6 inactivation. These results suggest STAT6 as a promising drug target for treatment of ADPKD...
  22. Simons M, Mlodzik M. Planar cell polarity signaling: from fly development to human disease. Annu Rev Genet. 2008;42:517-40 pubmed publisher
    ..This review discusses new developments in the molecular understanding of PCP establishment in Drosophila and vertebrates; these developments are integrated with new evidence that links PCP signaling to human disease...
  23. Pandey P, Brors B, Srivastava P, Bott A, Boehn S, Groene H, et al. Microarray-based approach identifies microRNAs and their target functional patterns in polycystic kidney disease. BMC Genomics. 2008;9:624 pubmed publisher
    ..Although involvement of certain genes and transcriptional networks in PKD development has been shown, not much is known how they are regulated molecularly...
  24. Cook S, Collin G, Bronson R, Naggert J, Liu D, Akeson E, et al. A mouse model for Meckel syndrome type 3. J Am Soc Nephrol. 2009;20:753-64 pubmed publisher
    ..This mutant provides a mouse model for MKS3 and adds to the growing set of mammalian models essential for studying the role of the primary cilium in kidney function...
  25. Patel V, Hajarnis S, Williams D, Hunter R, Huynh D, Igarashi P. MicroRNAs regulate renal tubule maturation through modulation of Pkd1. J Am Soc Nephrol. 2012;23:1941-8 pubmed publisher
    ..In conclusion, miRNAs are essential for the maturation of renal tubules, and Pkd1 is a target of miR-200. These results also suggest that miRNAs may modulate PKD1 gene dosage and play a role in the initiation of cystogenesis...
  26. Wallace D. Cyclic AMP-mediated cyst expansion. Biochim Biophys Acta. 2011;1812:1291-300 pubmed publisher
    ..This article is part of a Special Issue entitled: Polycystic Kidney Disease...
  27. Shillingford J, Piontek K, Germino G, Weimbs T. Rapamycin ameliorates PKD resulting from conditional inactivation of Pkd1. J Am Soc Nephrol. 2010;21:489-97 pubmed publisher
    ..These data provide in vivo evidence that rapamycin is effective in a human-orthologous mouse model of PKD...
  28. Xu T, Wang N, Fu L, Ye C, Yu S, Mei C. Celecoxib inhibits growth of human autosomal dominant polycystic kidney cyst-lining epithelial cells through the VEGF/Raf/MAPK/ERK signaling pathway. Mol Biol Rep. 2012;39:7743-53 pubmed publisher
    ..CXB can inhibit proliferation, suppress cell cycle progression, and induce apoptosis in ADPKD cyst-lining epithelial cells through the inhibition of the VEGF/VEGFR-2/Raf-1/MAPK/ERK signaling pathway...
  29. Nagao S, Kugita M, Yoshihara D, Yamaguchi T. Animal models for human polycystic kidney disease. Exp Anim. 2012;61:477-88 pubmed
    ..Therefore, experimental animal models are indispensable for investigating molecular mechanisms of PKD onset and progression as well as potential therapeutic treatments...
  30. Wu M, Arcaro A, Varga Z, Vogetseder A, Le Hir M, Wuthrich R, et al. Pulse mTOR inhibitor treatment effectively controls cyst growth but leads to severe parenchymal and glomerular hypertrophy in rat polycystic kidney disease. Am J Physiol Renal Physiol. 2009;297:F1597-605 pubmed publisher
    ..Our data might have an impact when long-term treatment using mTOR inhibitors in patients with autosomal dominant polycystic kidney disease is being considered...
  31. Ko J, Park J. Mouse models of polycystic kidney disease induced by defects of ciliary proteins. BMB Rep. 2013;46:73-9 pubmed
    ..In this review, we will describe the general role of cilia in renal epithelial cells, and the relationship between ciliary defects and PKD. We also discuss mouse models of PKD related to ciliary defects based on recent studies...
  32. Shiba D, Yamaoka Y, Hagiwara H, Takamatsu T, Hamada H, Yokoyama T. Localization of Inv in a distinctive intraciliary compartment requires the C-terminal ninein-homolog-containing region. J Cell Sci. 2009;122:44-54 pubmed publisher
  33. Romaker D, Puetz M, Teschner S, Donauer J, Geyer M, Gerke P, et al. Increased expression of secreted frizzled-related protein 4 in polycystic kidneys. J Am Soc Nephrol. 2009;20:48-56 pubmed publisher
    ..Taken together, these observations suggest a potential role for SFRP4 in the pathogenesis of ADPKD...
  34. Tao Y, Zafar I, Kim J, Schrier R, Edelstein C. Caspase-3 gene deletion prolongs survival in polycystic kidney disease. J Am Soc Nephrol. 2008;19:749-55 pubmed publisher
  35. Lancaster M, Gleeson J. The primary cilium as a cellular signaling center: lessons from disease. Curr Opin Genet Dev. 2009;19:220-9 pubmed publisher
    ..New findings are quickly lending insight into the functions of this cellular extension that seems to be especially important in modulation of subcellular signaling cascades at various stages of development and adult homeostasis...
  36. Zafar I, Belibi F, He Z, Edelstein C. Long-term rapamycin therapy in the Han:SPRD rat model of polycystic kidney disease (PKD). Nephrol Dial Transplant. 2009;24:2349-53 pubmed publisher
    ..Autosomal dominant polycystic kidney disease (ADPKD) patients would likely require life-long treatment with rapamycin...
  37. Wang X, Ward C, Harris P, Torres V. Cyclic nucleotide signaling in polycystic kidney disease. Kidney Int. 2010;77:129-40 pubmed publisher
    ..This may account, in part, for increased cyclic nucleotide signaling in polycystic kidney disease and contribute substantially to disease progression...
  38. Nishio S, Tian X, Gallagher A, Yu Z, Patel V, Igarashi P, et al. Loss of oriented cell division does not initiate cyst formation. J Am Soc Nephrol. 2010;21:295-302 pubmed publisher
    ..In conclusion, loss of oriented cell division is a feature of Pkhd1 mutation and cyst formation, but it is neither sufficient to produce kidney cysts nor required to initiate cyst formation after mutation in Pkd1 or Pkd2...
  39. Dowdle W, Robinson J, Kneist A, Sirerol Piquer M, Frints S, Corbit K, et al. Disruption of a ciliary B9 protein complex causes Meckel syndrome. Am J Hum Genet. 2011;89:94-110 pubmed publisher
    ..Our data indicate that B9d1 is required for normal Hh signaling, ciliogenesis, and ciliary protein localization and that B9d1 and B9d2 are essential components of a B9 protein complex, disruption of which causes MKS...
  40. Wilson P. Apico-basal polarity in polycystic kidney disease epithelia. Biochim Biophys Acta. 2011;1812:1239-48 pubmed publisher
    ..This article is part of a Special Issue entitled: Polycystic Kidney Disease...
  41. Edelstein C. What is the role of tubular epithelial cell apoptosis in polycystic kidney disease (PKD)?. Cell Cycle. 2005;4:1550-4 pubmed
    ..Thus, there is evidence that both epithelial cell apoptosis and proliferation are dysregulated in ADPKD and may represent a general mechanism for cyst growth. ..
  42. Mahjoub M, Trapp M, Quarmby L. NIMA-related kinases defective in murine models of polycystic kidney diseases localize to primary cilia and centrosomes. J Am Soc Nephrol. 2005;16:3485-9 pubmed
    A key feature of the polycystic kidney diseases is aberrant cell proliferation, a consequence of dysfunctional ciliary signaling...
  43. Davenport J, Yoder B. An incredible decade for the primary cilium: a look at a once-forgotten organelle. Am J Physiol Renal Physiol. 2005;289:F1159-69 pubmed
    ..Research focused on addressing this issue will be of critical importance for a further understanding of how ciliary dysfunction can lead to such severe disease and developmental pathologies. ..
  44. Igarashi P, Somlo S. Polycystic kidney disease. J Am Soc Nephrol. 2007;18:1371-3 pubmed
  45. Abbott K, Agodoa L. Polycystic kidney disease at end-stage renal disease in the United States: patient characteristics and survival. Clin Nephrol. 2002;57:208-14 pubmed
  46. Gattone V, Wang X, Harris P, Torres V. Inhibition of renal cystic disease development and progression by a vasopressin V2 receptor antagonist. Nat Med. 2003;9:1323-6 pubmed
    The polycystic kidney diseases (PKDs) are a group of genetic disorders causing significant renal failure and death in children and adults. There are no effective treatments...
  47. Guay Woodford L. Murine models of polycystic kidney disease: molecular and therapeutic insights. Am J Physiol Renal Physiol. 2003;285:F1034-49 pubmed
  48. Roitbak T, Ward C, Harris P, Bacallao R, Ness S, Wandinger Ness A. A polycystin-1 multiprotein complex is disrupted in polycystic kidney disease cells. Mol Biol Cell. 2004;15:1334-46 pubmed
  49. Couillard M, Guillaume R, Tanji N, D AGATI V, Trudel M. c-myc-induced apoptosis in polycystic kidney disease is independent of FasL/Fas interaction. Cancer Res. 2002;62:2210-4 pubmed
    ..Our study proves that c-myc-induced apoptosis can be independent of the FasL/Fas pathway in vivo and implicates the existence of a novel c-myc-driven apoptotic pathway. ..
  50. Morishita Y, Matsuzaki T, Hara Chikuma M, Andoo A, Shimono M, Matsuki A, et al. Disruption of aquaporin-11 produces polycystic kidneys following vacuolization of the proximal tubule. Mol Cell Biol. 2005;25:7770-9 pubmed
    ..These data demonstrate that AQP11 is essential for the proximal tubular function. AQP11-null mice are a novel model for polycystic kidney diseases and will provide a new mechanism for cystogenesis.
  51. Tao Y, Kim J, Faubel S, Wu J, Falk S, Schrier R, et al. Caspase inhibition reduces tubular apoptosis and proliferation and slows disease progression in polycystic kidney disease. Proc Natl Acad Sci U S A. 2005;102:6954-9 pubmed
  52. Yoder B. Role of primary cilia in the pathogenesis of polycystic kidney disease. J Am Soc Nephrol. 2007;18:1381-8 pubmed
  53. Simons M, Walz G. Polycystic kidney disease: cell division without a c(l)ue?. Kidney Int. 2006;70:854-64 pubmed
    ..Failure to communicate this spatial information may condemn the tubular epithelial cells to proliferate and to form cysts...
  54. Taha D, Barbar M, Kanaan H, Williamson Balfe J. Neonatal diabetes mellitus, congenital hypothyroidism, hepatic fibrosis, polycystic kidneys, and congenital glaucoma: a new autosomal recessive syndrome?. Am J Med Genet A. 2003;122A:269-73 pubmed
    ..Mutation analysis for several candidate genes is warranted...
  55. Fain P, McFann K, Taylor M, Tison M, Johnson A, Reed B, et al. Modifier genes play a significant role in the phenotypic expression of PKD1. Kidney Int. 2005;67:1256-67 pubmed
    ..There are significant familial phenotype differences; but it is not clear if this is due to differences in PKD1 mutations, differences in genetic background, or both...
  56. Mochizuki E, Fukuta K, Tada T, Harada T, Watanabe N, Matsuo S, et al. Fish mesonephric model of polycystic kidney disease in medaka (Oryzias latipes) pc mutant. Kidney Int. 2005;68:23-34 pubmed
    ..Here, we report a medaka (Oryzias latipes) mutant that develops numerous cysts in the kidney in adulthood fish in an autosomal-recessive manner as a mesonephric model of PKD...
  57. Leuenroth S, Okuhara D, Shotwell J, Markowitz G, Yu Z, Somlo S, et al. Triptolide is a traditional Chinese medicine-derived inhibitor of polycystic kidney disease. Proc Natl Acad Sci U S A. 2007;104:4389-94 pubmed
    ..We anticipate that small molecule induction of PC2-dependent calcium release is likely to be a valid therapeutic strategy for ADPKD...
  58. Bernhardt W, Wiesener M, Weidemann A, Schmitt R, Weichert W, Lechler P, et al. Involvement of hypoxia-inducible transcription factors in polycystic kidney disease. Am J Pathol. 2007;170:830-42 pubmed
    ..In conclusion, HIF accumulation in human and rat PKD seems to be responsible for increased EPO production and pericystic hypervascularity and may have an impact on progression of PKD...
  59. Chiu M, Johnson T, Woolf A, Dahm Vicker E, Long D, Guay Woodford L, et al. Galectin-3 associates with the primary cilium and modulates cyst growth in congenital polycystic kidney disease. Am J Pathol. 2006;169:1925-38 pubmed
    ..These data raise the possibility that galectin-3 may act as a natural brake on cystogenesis in cpk mice, perhaps via ciliary roles...
  60. Nauli S, Zhou J. Polycystins and mechanosensation in renal and nodal cilia. Bioessays. 2004;26:844-56 pubmed
  61. Pan J, Wang Q, Snell W. Cilium-generated signaling and cilia-related disorders. Lab Invest. 2005;85:452-63 pubmed
  62. Roume J, Ville Y. Prenatal diagnosis of genetic renal diseases: breaking the code. Ultrasound Obstet Gynecol. 2004;24:10-8 pubmed
  63. Lin F, Hiesberger T, Cordes K, Sinclair A, Goldstein L, Somlo S, et al. Kidney-specific inactivation of the KIF3A subunit of kinesin-II inhibits renal ciliogenesis and produces polycystic kidney disease. Proc Natl Acad Sci U S A. 2003;100:5286-91 pubmed
    ..Most generally, the phenotype of Kif3a mutant mice suggests a role for primary cilia in the maintenance of lumen-forming epithelial differentiation...
  64. Magenheimer B, St John P, Isom K, Abrahamson D, De Lisle R, Wallace D, et al. Early embryonic renal tubules of wild-type and polycystic kidney disease kidneys respond to cAMP stimulation with cystic fibrosis transmembrane conductance regulator/Na(+),K(+),2Cl(-) Co-transporter-dependent cystic dilation. J Am Soc Nephrol. 2006;17:3424-37 pubmed