Published in Science on May 27, 1994
Chlamydomonas IFT88 and its mouse homologue, polycystic kidney disease gene tg737, are required for assembly of cilia and flagella. J Cell Biol (2000) 7.04
Gli2 and Gli3 localize to cilia and require the intraflagellar transport protein polaris for processing and function. PLoS Genet (2005) 6.15
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 (2003) 5.24
The intraflagellar transport protein IFT20 is associated with the Golgi complex and is required for cilia assembly. Mol Biol Cell (2006) 4.18
Candidate exome capture identifies mutation of SDCCAG8 as the cause of a retinal-renal ciliopathy. Nat Genet (2010) 3.99
The intraflagellar transport protein, IFT88, is essential for vertebrate photoreceptor assembly and maintenance. J Cell Biol (2002) 3.85
A large-scale insertional mutagenesis screen in zebrafish. Genes Dev (1999) 3.57
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Loss of C. elegans BBS-7 and BBS-8 protein function results in cilia defects and compromised intraflagellar transport. Genes Dev (2004) 3.21
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Polaris, a protein involved in left-right axis patterning, localizes to basal bodies and cilia. Mol Biol Cell (2001) 2.90
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Pancreatic cancer and precursor pancreatic intraepithelial neoplasia lesions are devoid of primary cilia. Cancer Res (2009) 2.43
A transcriptional network in polycystic kidney disease. EMBO J (2004) 2.21
Cystin, a novel cilia-associated protein, is disrupted in the cpk mouse model of polycystic kidney disease. J Clin Invest (2002) 2.07
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Functional redundancy of the B9 proteins and nephrocystins in Caenorhabditis elegans ciliogenesis. Mol Biol Cell (2008) 1.59
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The Talpid3 gene (KIAA0586) encodes a centrosomal protein that is essential for primary cilia formation. Development (2009) 1.45
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Primary cilia regulate Shh activity in the control of molar tooth number. Development (2009) 1.31
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Biliary and pancreatic dysgenesis in mice harboring a mutation in Pkhd1. Am J Pathol (2008) 1.17
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Photoreceptor IFT complexes containing chaperones, guanylyl cyclase 1 and rhodopsin. Traffic (2009) 1.16
Immunolocalization of ion transport proteins in human autosomal dominant polycystic kidney epithelial cells. Proc Natl Acad Sci U S A (1996) 1.15
Cep70 and Cep131 contribute to ciliogenesis in zebrafish embryos. BMC Cell Biol (2009) 1.14
Kinesin-II is required for flagellar sensory transduction during fertilization in Chlamydomonas. Mol Biol Cell (2002) 1.13
The primary cilium coordinates early cardiogenesis and hedgehog signaling in cardiomyocyte differentiation. J Cell Sci (2009) 1.12
The hydrocephalus inducing gene product, Hydin, positions axonemal central pair microtubules. BMC Biol (2007) 1.11
The exocyst protein Sec10 interacts with Polycystin-2 and knockdown causes PKD-phenotypes. PLoS Genet (2011) 1.11
Cftr controls lumen expansion and function of Kupffer's vesicle in zebrafish. Development (2013) 1.09
Differential rescue of the renal and hepatic disease in an autosomal recessive polycystic kidney disease mouse mutant. A new model to study the liver lesion. Am J Pathol (1997) 1.05
Is left-right asymmetry a form of planar cell polarity? Development (2009) 1.04
Primary cilia regulate branching morphogenesis during mammary gland development. Curr Biol (2010) 1.03
Polycystic kidney disease in SBM transgenic mice: role of c-myc in disease induction and progression. Am J Pathol (1998) 1.00
Cystic diseases of the kidney: ciliary dysfunction and cystogenic mechanisms. Pediatr Nephrol (2010) 0.98
Hepato-renal pathology in pkd2ws25/- mice, an animal model of autosomal dominant polycystic kidney disease. Am J Pathol (2010) 0.97
Keeping the balance between proliferation and differentiation: the primary cilium. Curr Genomics (2011) 0.96
Crystal structures of IFT70/52 and IFT52/46 provide insight into intraflagellar transport B core complex assembly. J Cell Biol (2014) 0.96
The role for HNF-1beta-targeted collectrin in maintenance of primary cilia and cell polarity in collecting duct cells. PLoS One (2007) 0.96
Primary cilia: highly sophisticated biological sensors. Sensors (Basel) (2009) 0.95
Intraflagellar transport proteins 172, 80, 57, 54, 38, and 20 form a stable tubulin-binding IFT-B2 complex. EMBO J (2016) 0.94
Collecting duct cells that lack normal cilia have mislocalized vasopressin-2 receptors. Am J Physiol Renal Physiol (2011) 0.92
Situs inversus and ciliary abnormalities: 20 years later, what is the connection? Cilia (2015) 0.92
Primary cilia dynamics instruct tissue patterning and repair of corneal endothelium. Proc Natl Acad Sci U S A (2011) 0.91
Primary cilia modulate Ihh signal transduction in response to hydrostatic loading of growth plate chondrocytes. Bone (2011) 0.90
Developmental stalling and organ-autonomous regulation of morphogenesis. Proc Natl Acad Sci U S A (2011) 0.89
Prefoldin 5 is required for normal sensory and neuronal development in a murine model. J Biol Chem (2010) 0.89
Oval cell proliferation associated with the murine insertional mutation TgN737Rpw. Am J Pathol (1996) 0.88
Pathophysiology of childhood polycystic kidney diseases: new insights into disease-specific therapy. Pediatr Res (2013) 0.84
A mechanistic approach to inherited polycystic kidney disease. Pediatr Nephrol (2005) 0.84
New insights into ciliary function: kidney cysts and photoreceptors. Proc Natl Acad Sci U S A (2003) 0.84
Isolation and characterization of liver epithelial cell lines from wild-type and mutant TgN737Rpw mice. Am J Pathol (1997) 0.83
Role of genetic modifiers in an orthologous rat model of ARPKD. Physiol Genomics (2012) 0.83
Increased Na+/H+ exchanger activity on the apical surface of a cilium-deficient cortical collecting duct principal cell model of polycystic kidney disease. Am J Physiol Cell Physiol (2012) 0.82
Interleukin-1β sequesters hypoxia inducible factor 2α to the primary cilium. Cilia (2013) 0.81
IFT80 is essential for chondrocyte differentiation by regulating Hedgehog and Wnt signaling pathways. Exp Cell Res (2013) 0.81
Heterotrimeric kinesin-2 (KIF3) mediates transition zone and axoneme formation of mouse photoreceptors. J Biol Chem (2015) 0.80
A mutation in the mouse ttc26 gene leads to impaired hedgehog signaling. PLoS Genet (2014) 0.80
Structural and Functional Recovery of Sensory Cilia in C. elegans IFT Mutants upon Aging. PLoS Genet (2016) 0.79
Intraflagellar transport is essential for mammalian spermiogenesis but is absent in mature sperm. Mol Biol Cell (2015) 0.78
A Cilia Independent Role of Ift88/Polaris during Cell Migration. PLoS One (2015) 0.78
Unravelling the pathogenesis of cystic kidney diseases. Arch Dis Child (1995) 0.78
Cilia/Ift protein and motor -related bone diseases and mouse models. Front Biosci (Landmark Ed) (2015) 0.78
Epidermal growth factor-induced proliferation of collecting duct cells from Oak Ridge polycystic kidney mice involves activation of Na+/H+ exchanger. Am J Physiol Cell Physiol (2014) 0.77
Transport, cilia, and PKD: must we in (cyst) on interrelationships? Focus on "Increased Na+/H+ exchanger activity on the apical surface of a cilium-deficient cortical collecting duct principal cell model of polycystic kidney disease". Am J Physiol Cell Physiol (2012) 0.77
Aberrant expression of laminin-332 promotes cell proliferation and cyst growth in ARPKD. Am J Physiol Renal Physiol (2013) 0.77
Mouse models of polycystic kidney disease induced by defects of ciliary proteins. BMB Rep (2013) 0.76
Identification of the human CYS1 gene and candidate gene analysis in Boichis disease. Pediatr Nephrol (2003) 0.76
Cilia gene mutations cause atrioventricular septal defects by multiple mechanisms. Hum Mol Genet (2016) 0.76
Unique spatiotemporal requirements for intraflagellar transport genes during forebrain development. PLoS One (2017) 0.75
Case study: polycystic livers in a transgenic mouse line. Comp Med (2014) 0.75
The fate of bone marrow-derived cells carrying a Polycystic Kidney Disease mutation in the genetically normal kidney. BMC Nephrol (2012) 0.75
Cilia and polycystic kidney disease, kith and kin. Birth Defects Res C Embryo Today (2014) 0.75
CILIA: before and after. Cilia (2017) 0.75
The CEP19-RABL2 GTPase Complex Binds IFT-B to Initiate Intraflagellar Transport at the Ciliary Base. Dev Cell (2017) 0.75
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