Published in Proc Natl Acad Sci U S A on June 13, 2011
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International Union of Basic and Clinical Pharmacology. CI. Structures and Small Molecule Modulators of Mammalian Adenylyl Cyclases. Pharmacol Rev (2017) 0.83
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Polycystins and cellular Ca2+ signaling. Cell Mol Life Sci (2012) 0.78
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Developmental signaling: does it bridge the gap between cilia dysfunction and renal cystogenesis? Birth Defects Res C Embryo Today (2014) 0.78
The cAMP Signaling Pathway and Direct Protein Kinase A Phosphorylation Regulate Polycystin-2 (TRPP2) Channel Function. J Biol Chem (2015) 0.77
The Roles of Primary cilia in Polycystic Kidney Disease. AIMS Mol Sci (2015) 0.77
Mouse models of polycystic kidney disease induced by defects of ciliary proteins. BMB Rep (2013) 0.76
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Adenylyl Cyclase 5 links changes in calcium homeostasis to cAMP-dependent cyst growth in Polycystic Liver Disease. J Hepatol (2016) 0.75
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The mechanosensitive BKα/β1 channel localizes to cilia of principal cells in rabbit cortical collecting duct (CCD). Am J Physiol Renal Physiol (2016) 0.75
Loss of Glis2/NPHP7 causes kidney epithelial cell senescence and suppresses cyst growth in the Kif3a mouse model of cystic kidney disease. Kidney Int (2016) 0.75
Type 3 adenylyl cyclase: a key enzyme mediating the cAMP signaling in neuronal cilia. Int J Physiol Pathophysiol Pharmacol (2016) 0.75
A cAMP and CREB-mediated feed-forward mechanism regulates GSK3β in polycystic kidney disease. J Mol Cell Biol (2016) 0.75
Tubby family proteins are adapters for ciliary trafficking of integral membrane proteins. J Cell Biol (2017) 0.75
TGR5 contributes to hepatic cystogenesis in rodents with polycystic liver diseases via cAMP/Gαs signaling. Hepatology (2017) 0.75
Polycystins 1 and 2 mediate mechanosensation in the primary cilium of kidney cells. Nat Genet (2003) 13.33
The ciliopathies: an emerging class of human genetic disorders. Annu Rev Genomics Hum Genet (2006) 9.56
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) 4.47
Inhibition of renal cystic disease development and progression by a vasopressin V2 receptor antagonist. Nat Med (2003) 3.78
The primary cilium as a complex signaling center. Curr Biol (2009) 3.73
Genetics and pathogenesis of polycystic kidney disease. J Am Soc Nephrol (2002) 3.52
Randomized clinical trial of long-acting somatostatin for autosomal dominant polycystic kidney and liver disease. J Am Soc Nephrol (2010) 3.47
Acute kidney injury and aberrant planar cell polarity induce cyst formation in mice lacking renal cilia. Hum Mol Genet (2008) 3.14
Mechanisms of Disease: autosomal dominant and recessive polycystic kidney diseases. Nat Clin Pract Nephrol (2006) 2.95
Polycystin-2 traffics to cilia independently of polycystin-1 by using an N-terminal RVxP motif. J Cell Sci (2006) 2.72
Cholangiocyte cilia detect changes in luminal fluid flow and transmit them into intracellular Ca2+ and cAMP signaling. Gastroenterology (2006) 2.43
Cyclic AMP activates B-Raf and ERK in cyst epithelial cells from autosomal-dominant polycystic kidneys. Kidney Int (2003) 2.32
A transcriptional network in polycystic kidney disease. EMBO J (2004) 2.21
A physiological view of the primary cilium. Annu Rev Physiol (2005) 2.20
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Molecular advances in autosomal dominant polycystic kidney disease. Adv Chronic Kidney Dis (2010) 1.77
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Mutation of hepatocyte nuclear factor-1beta inhibits Pkhd1 gene expression and produces renal cysts in mice. J Clin Invest (2004) 1.67
PKD2 functions as an epidermal growth factor-activated plasma membrane channel. Mol Cell Biol (2005) 1.64
Cholangiocyte primary cilia are chemosensory organelles that detect biliary nucleotides via P2Y12 purinergic receptors. Am J Physiol Gastrointest Liver Physiol (2008) 1.56
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) 1.50
Roles of HNF-1beta in kidney development and congenital cystic diseases. Kidney Int (2005) 1.36
Compartmentalisation of cAMP and Ca(2+) signals. Curr Opin Cell Biol (2002) 1.33
Mutations of HNF-1beta inhibit epithelial morphogenesis through dysregulation of SOCS-3. Proc Natl Acad Sci U S A (2007) 1.20
Deciliation is associated with dramatic remodeling of epithelial cell junctions and surface domains. Mol Biol Cell (2008) 1.15
Vasopressin receptor-mediated functional signaling pathway in primary cilia of renal epithelial cells. Am J Physiol Renal Physiol (2008) 1.14
Renal epithelial fluid secretion and cyst growth: the role of cyclic AMP. FASEB J (1989) 1.13
Cyclic nucleotide signaling in polycystic kidney disease. Kidney Int (2009) 1.09
The polycystin 1-C-terminal fragment stimulates ERK-dependent spreading of renal epithelial cells. J Biol Chem (2006) 1.05
The effect of caffeine on renal epithelial cells from patients with autosomal dominant polycystic kidney disease. J Am Soc Nephrol (2002) 1.01
[Ca2+]i reduction increases cellular proliferation and apoptosis in vascular smooth muscle cells: relevance to the ADPKD phenotype. Circ Res (2005) 1.01
Strategies to inhibit cyst formation in ADPKD. Clin J Am Soc Nephrol (2008) 0.99
Impaired water reabsorption in mice deficient in the type VI adenylyl cyclase (AC6). FEBS Lett (2010) 0.99
Orangutan cultures and the evolution of material culture. Science (2003) 6.06
Long noncoding RNA HOTAIR regulates polycomb-dependent chromatin modification and is associated with poor prognosis in colorectal cancers. Cancer Res (2011) 6.02
Two populations of node monocilia initiate left-right asymmetry in the mouse. Cell (2003) 4.73
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) 4.47
Polycystin-2 is an intracellular calcium release channel. Nat Cell Biol (2002) 4.37
Regulation of myocardial contractility and cell size by distinct PI3K-PTEN signaling pathways. Cell (2002) 4.35
Defective planar cell polarity in polycystic kidney disease. Nat Genet (2005) 4.31
Loss of Fat4 disrupts PCP signaling and oriented cell division and leads to cystic kidney disease. Nat Genet (2008) 4.28
Electrical signals control wound healing through phosphatidylinositol-3-OH kinase-gamma and PTEN. Nature (2006) 4.09
Hepatocyte-specific Pten deficiency results in steatohepatitis and hepatocellular carcinomas. J Clin Invest (2004) 3.92
Notch-RBP-J signaling is involved in cell fate determination of marginal zone B cells. Nat Immunol (2002) 3.75
Primary cilia regulate mTORC1 activity and cell size through Lkb1. Nat Cell Biol (2010) 3.55
Genetics and pathogenesis of polycystic kidney disease. J Am Soc Nephrol (2002) 3.52
AKT2 is essential to maintain podocyte viability and function during chronic kidney disease. Nat Med (2013) 3.41
Epithelial-specific Cre/lox recombination in the developing kidney and genitourinary tract. J Am Soc Nephrol (2002) 3.28
PKHD1, the polycystic kidney and hepatic disease 1 gene, encodes a novel large protein containing multiple immunoglobulin-like plexin-transcription-factor domains and parallel beta-helix 1 repeats. Am J Hum Genet (2002) 3.21
Control of cell polarity and motility by the PtdIns(3,4,5)P3 phosphatase SHIP1. Nat Cell Biol (2006) 3.19
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) 3.18
Acute kidney injury and aberrant planar cell polarity induce cyst formation in mice lacking renal cilia. Hum Mol Genet (2008) 3.14
Nitrogen uptake, assimilation and remobilization in plants: challenges for sustainable and productive agriculture. Ann Bot (2010) 3.11
Effective treatment of an orthologous model of autosomal dominant polycystic kidney disease. Nat Med (2004) 3.09
Intrarenal cells, not bone marrow-derived cells, are the major source for regeneration in postischemic kidney. J Clin Invest (2005) 2.94
Exosome release of β-catenin: a novel mechanism that antagonizes Wnt signaling. J Cell Biol (2010) 2.85
Wnt9b signaling regulates planar cell polarity and kidney tubule morphogenesis. Nat Genet (2009) 2.84
Polycystin-2 traffics to cilia independently of polycystin-1 by using an N-terminal RVxP motif. J Cell Sci (2006) 2.72
Loss of cilia suppresses cyst growth in genetic models of autosomal dominant polycystic kidney disease. Nat Genet (2013) 2.71
Targeted inactivation of fh1 causes proliferative renal cyst development and activation of the hypoxia pathway. Cancer Cell (2007) 2.69
Evaluation of urine biomarkers of kidney injury in polycystic kidney disease. Kidney Int (2012) 2.39
Curcumin, a major constituent of turmeric, corrects cystic fibrosis defects. Science (2004) 2.39
Enhanced PIP3 signaling in POMC neurons causes KATP channel activation and leads to diet-sensitive obesity. J Clin Invest (2006) 2.38
DCDC2 is associated with reading disability and modulates neuronal development in the brain. Proc Natl Acad Sci U S A (2005) 2.35
Bile system morphogenesis defects and liver dysfunction upon targeted deletion of HNF1beta. Development (2002) 2.27
Kidney-targeted Birt-Hogg-Dube gene inactivation in a mouse model: Erk1/2 and Akt-mTOR activation, cell hyperproliferation, and polycystic kidneys. J Natl Cancer Inst (2008) 2.25
Multiple renal cysts, urinary concentration defects, and pulmonary emphysematous changes in mice lacking TAZ. Am J Physiol Renal Physiol (2008) 2.24
Cyst formation and activation of the extracellular regulated kinase pathway after kidney specific inactivation of Pkd1. Hum Mol Genet (2008) 2.23
A transcriptional network in polycystic kidney disease. EMBO J (2004) 2.21
Loss of oriented cell division does not initiate cyst formation. J Am Soc Nephrol (2009) 2.20
The PTEN/PI3K pathway governs normal vascular development and tumor angiogenesis. Genes Dev (2005) 2.18
Major issues and challenges of influenza pandemic preparedness in developing countries. Emerg Infect Dis (2008) 2.16
Hematopoietic stem cells contribute to the regeneration of renal tubules after renal ischemia-reperfusion injury in mice. J Am Soc Nephrol (2003) 2.12
Loss of NFAT5 results in renal atrophy and lack of tonicity-responsive gene expression. Proc Natl Acad Sci U S A (2004) 2.12
Olfactory receptor responding to gut microbiota-derived signals plays a role in renin secretion and blood pressure regulation. Proc Natl Acad Sci U S A (2013) 2.10
Mechanical stimuli induce cleavage and nuclear translocation of the polycystin-1 C terminus. J Clin Invest (2004) 2.00
AMP-activated protein kinase regulates the assembly of epithelial tight junctions. Proc Natl Acad Sci U S A (2006) 1.98
High prevalence of obstructive sleep apnea and its association with renal function among nondialysis chronic kidney disease patients in Japan: a cross-sectional study. Clin J Am Soc Nephrol (2011) 1.98
Enterovirus 68 among children with severe acute respiratory infection, the Philippines. Emerg Infect Dis (2011) 1.96
Mutations in SEC63 cause autosomal dominant polycystic liver disease. Nat Genet (2004) 1.96
Muscle-specific Pten deletion protects against insulin resistance and diabetes. Mol Cell Biol (2005) 1.93
Canonical Wnt9b signaling balances progenitor cell expansion and differentiation during kidney development. Development (2011) 1.92
Functional expression of the olfactory signaling system in the kidney. Proc Natl Acad Sci U S A (2009) 1.90
Smad2 protects against TGF-beta/Smad3-mediated renal fibrosis. J Am Soc Nephrol (2010) 1.89
Pten controls lung morphogenesis, bronchioalveolar stem cells, and onset of lung adenocarcinomas in mice. J Clin Invest (2007) 1.86
A classification of ductal plate malformations based on distinct pathogenic mechanisms of biliary dysmorphogenesis. Hepatology (2011) 1.84
The uptake and intracellular fate of PLGA nanoparticles in epithelial cells. Biomaterials (2009) 1.82
Conditional loss of PTEN leads to testicular teratoma and enhances embryonic germ cell production. Development (2003) 1.80
How megalin finds its way: identification of a novel apical sorting motif. Focus on "Identification of an apical sorting determinant in the cytoplasmic tail of megalin". Am J Physiol Cell Physiol (2003) 1.78
Molecular advances in autosomal dominant polycystic kidney disease. Adv Chronic Kidney Dis (2010) 1.77
Cystic renal neoplasia following conditional inactivation of apc in mouse renal tubular epithelium. J Biol Chem (2004) 1.77
Calcium-pump inhibitors induce functional surface expression of Delta F508-CFTR protein in cystic fibrosis epithelial cells. Nat Med (2002) 1.75
Triptolide is a traditional Chinese medicine-derived inhibitor of polycystic kidney disease. Proc Natl Acad Sci U S A (2007) 1.75
Advances in the pathogenesis and treatment of polycystic kidney disease. Curr Opin Nephrol Hypertens (2009) 1.73
A minimal Ksp-cadherin promoter linked to a green fluorescent protein reporter gene exhibits tissue-specific expression in the developing kidney and genitourinary tract. J Am Soc Nephrol (2002) 1.72
Inflammasome-activating nanoparticles as modular systems for optimizing vaccine efficacy. Vaccine (2009) 1.72
Altered trafficking and stability of polycystins underlie polycystic kidney disease. J Clin Invest (2014) 1.71
Hepatic stem-like phenotype and interplay of Wnt/beta-catenin and Myc signaling in aggressive childhood liver cancer. Cancer Cell (2008) 1.70
Spectrum of mutations in the gene for autosomal recessive polycystic kidney disease (ARPKD/PKHD1). J Am Soc Nephrol (2003) 1.70
Activating AMP-activated protein kinase (AMPK) slows renal cystogenesis. Proc Natl Acad Sci U S A (2011) 1.69
Retracted Enhanced insulin sensitivity, energy expenditure and thermogenesis in adipose-specific Pten suppression in mice. Nat Med (2004) 1.69
T-cell factor/β-catenin activity is suppressed in two different models of autosomal dominant polycystic kidney disease. Kidney Int (2011) 1.69
Early onset of neoplasia in the prostate and skin of mice with tissue-specific deletion of Pten. Proc Natl Acad Sci U S A (2004) 1.67
Mutation of hepatocyte nuclear factor-1beta inhibits Pkhd1 gene expression and produces renal cysts in mice. J Clin Invest (2004) 1.67
Kidney injury molecule-1 expression in murine polycystic kidney disease. Am J Physiol Renal Physiol (2002) 1.64
Genetic basis of prune belly syndrome: screening for HNF1β gene. J Urol (2011) 1.64
Polycystin-1 C-terminal tail associates with beta-catenin and inhibits canonical Wnt signaling. Hum Mol Genet (2008) 1.62
Calcium dependence of polycystin-2 channel activity is modulated by phosphorylation at Ser812. J Biol Chem (2004) 1.60
Anterior paraclinoid aneurysms. J Neurosurg (2002) 1.59
The cell biology of polycystic kidney disease. J Cell Biol (2010) 1.58
Intragenic motifs regulate the transcriptional complexity of Pkhd1/PKHD1. J Mol Med (Berl) (2014) 1.56
Small-molecule CFTR inhibitors slow cyst growth in polycystic kidney disease. J Am Soc Nephrol (2008) 1.56
Regulation of the MDM2-P53 pathway and tumor growth by PICT1 via nucleolar RPL11. Nat Med (2011) 1.52
Mammalian phosphoinositide kinases and phosphatases. Prog Lipid Res (2009) 1.48
Mutations in PRKCSH cause isolated autosomal dominant polycystic liver disease. Am J Hum Genet (2003) 1.47
The aquaporin-2 water channel in autosomal dominant primary nocturnal enuresis. J Urol (2002) 1.46
Trans-heterozygous Pkd1 and Pkd2 mutations modify expression of polycystic kidney disease. Hum Mol Genet (2002) 1.44
Autophagy plays a critical role in kidney tubule maintenance, aging and ischemia-reperfusion injury. Autophagy (2012) 1.44
A mitotic transcriptional switch in polycystic kidney disease. Nat Med (2009) 1.42