Published in Cell on April 19, 2002
Ciliopathies. N Engl J Med (2011) 6.28
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
Molecular signatures of proliferation and quiescence in hematopoietic stem cells. PLoS Biol (2004) 3.94
Nephronophthisis: disease mechanisms of a ciliopathy. J Am Soc Nephrol (2008) 2.66
Cleavage of polycystin-1 requires the receptor for egg jelly domain and is disrupted by human autosomal-dominant polycystic kidney disease 1-associated mutations. Proc Natl Acad Sci U S A (2002) 2.29
Sirolimus reduces polycystic liver volume in ADPKD patients. J Am Soc Nephrol (2008) 2.26
A transcriptional network in polycystic kidney disease. EMBO J (2004) 2.21
Primary cilia and signaling pathways in mammalian development, health and disease. Nephron Physiol (2009) 2.09
Mechanical stimuli induce cleavage and nuclear translocation of the polycystin-1 C terminus. J Clin Invest (2004) 2.00
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TAZ promotes PC2 degradation through a SCFbeta-Trcp E3 ligase complex. Mol Cell Biol (2007) 1.86
Loss of p21 permits carcinogenesis from chronically damaged liver and kidney epithelial cells despite unchecked apoptosis. Cancer Cell (2008) 1.75
Triptolide is a traditional Chinese medicine-derived inhibitor of polycystic kidney disease. Proc Natl Acad Sci U S A (2007) 1.75
Hyperproliferation of PKD1 cystic cells is induced by insulin-like growth factor-1 activation of the Ras/Raf signalling system. Kidney Int (2007) 1.73
Pkd1 inactivation induced in adulthood produces focal cystic disease. J Am Soc Nephrol (2008) 1.65
The cell biology of polycystic kidney disease. J Cell Biol (2010) 1.58
Polycystin-1 regulates extracellular signal-regulated kinase-dependent phosphorylation of tuberin to control cell size through mTOR and its downstream effectors S6K and 4EBP1. Mol Cell Biol (2009) 1.58
A novel mouse model reveals that polycystin-1 deficiency in ependyma and choroid plexus results in dysfunctional cilia and hydrocephalus. PLoS One (2009) 1.51
Polycystin-1 induces cell migration by regulating phosphatidylinositol 3-kinase-dependent cytoskeletal rearrangements and GSK3beta-dependent cell cell mechanical adhesion. Mol Biol Cell (2007) 1.47
Pkd1 regulates immortalized proliferation of renal tubular epithelial cells through p53 induction and JNK activation. J Clin Invest (2005) 1.46
Src inhibition ameliorates polycystic kidney disease. J Am Soc Nephrol (2008) 1.43
A mitotic transcriptional switch in polycystic kidney disease. Nat Med (2009) 1.42
Regulation of ryanodine receptor-dependent calcium signaling by polycystin-2. Proc Natl Acad Sci U S A (2007) 1.41
A polycystin-1 multiprotein complex is disrupted in polycystic kidney disease cells. Mol Biol Cell (2004) 1.39
Physiology and pathophysiology of the vasopressin-regulated renal water reabsorption. Pflugers Arch (2008) 1.37
Polycystins: polymodal receptor/ion-channel cellular sensors. Pflugers Arch (2005) 1.37
The versatile nature of the calcium-permeable cation channel TRPP2. EMBO Rep (2006) 1.34
Polycystin-1 induces resistance to apoptosis through the phosphatidylinositol 3-kinase/Akt signaling pathway. J Am Soc Nephrol (2006) 1.23
Cell biology of polycystin-2. Cell Signal (2006) 1.21
Loss of polycystin-1 causes centrosome amplification and genomic instability. Hum Mol Genet (2008) 1.20
Two N-myristoyltransferase isozymes play unique roles in protein myristoylation, proliferation, and apoptosis. Mol Cancer Res (2005) 1.20
Mutations of HNF-1beta inhibit epithelial morphogenesis through dysregulation of SOCS-3. Proc Natl Acad Sci U S A (2007) 1.20
Pkd1 haploinsufficiency increases renal damage and induces microcyst formation following ischemia/reperfusion. J Am Soc Nephrol (2009) 1.19
The nanomechanics of polycystin-1 extracellular region. J Biol Chem (2005) 1.18
Glis3 is associated with primary cilia and Wwtr1/TAZ and implicated in polycystic kidney disease. Mol Cell Biol (2009) 1.18
A polycystin-2 (TRPP2) dimerization domain essential for the function of heteromeric polycystin complexes. EMBO J (2010) 1.18
At the crossroads of homoeostasis and disease: roles of the PACS proteins in membrane traffic and apoptosis. Biochem J (2009) 1.15
Polycystin-1 regulates STAT activity by a dual mechanism. Proc Natl Acad Sci U S A (2011) 1.15
Sirtuin 1 inhibition delays cyst formation in autosomal-dominant polycystic kidney disease. J Clin Invest (2013) 1.14
The dynamic cilium in human diseases. Pathogenetics (2009) 1.13
p21 is decreased in polycystic kidney disease and leads to increased epithelial cell cycle progression: roscovitine augments p21 levels. BMC Nephrol (2007) 1.11
miR-17~92 miRNA cluster promotes kidney cyst growth in polycystic kidney disease. Proc Natl Acad Sci U S A (2013) 1.10
Polycystin-dependent fluid flow sensing targets histone deacetylase 5 to prevent the development of renal cysts. Development (2010) 1.10
Dose-dependent effects of sirolimus on mTOR signaling and polycystic kidney disease. J Am Soc Nephrol (2012) 1.10
Emerging evidence of a link between the polycystins and the mTOR pathways. Pathogenetics (2009) 1.09
Pyrimethamine inhibits adult polycystic kidney disease by modulating STAT signaling pathways. Hum Mol Genet (2011) 1.09
Fish and frogs: models for vertebrate cilia signaling. Front Biosci (2008) 1.08
Role of the polycytin-primary cilia complex in bone development and mechanosensing. Ann N Y Acad Sci (2010) 1.07
Pkd2 dosage influences cellular repair responses following ischemia-reperfusion injury. Am J Pathol (2009) 1.07
Growth of cranial synchondroses and sutures requires polycystin-1. Dev Biol (2008) 1.06
Genetic podocyte lineage reveals progressive podocytopenia with parietal cell hyperplasia in a murine model of cellular/collapsing focal segmental glomerulosclerosis. Am J Pathol (2009) 1.05
TRPpathies. J Physiol (2006) 1.04
Polycystic kidney disease: pathogenesis and potential therapies. Biochim Biophys Acta (2010) 1.02
Neutrophil gelatinase-associated lipocalin suppresses cyst growth by Pkd1 null cells in vitro and in vivo. Kidney Int (2008) 1.02
Systems biology approach to identify transcriptome reprogramming and candidate microRNA targets during the progression of polycystic kidney disease. BMC Syst Biol (2011) 1.02
Polycystic kidney diseases: from molecular discoveries to targeted therapeutic strategies. Cell Mol Life Sci (2008) 1.01
A polycystin-centric view of cyst formation and disease: the polycystins revisited. Kidney Int (2015) 1.01
Epidermal growth factor-mediated proliferation and sodium transport in normal and PKD epithelial cells. Biochim Biophys Acta (2010) 1.00
The role of the cilium in normal and abnormal cell cycles: emphasis on renal cystic pathologies. Cell Mol Life Sci (2012) 0.99
Cystic diseases of the kidney: ciliary dysfunction and cystogenic mechanisms. Pediatr Nephrol (2010) 0.98
Polycystin-1 interacts with inositol 1,4,5-trisphosphate receptor to modulate intracellular Ca2+ signaling with implications for polycystic kidney disease. J Biol Chem (2009) 0.96
Mating worms and the cystic kidney: Caenorhabditis elegans as a model for renal disease. Pediatr Nephrol (2005) 0.96
Keeping the balance between proliferation and differentiation: the primary cilium. Curr Genomics (2011) 0.96
The polycystic kidney disease 1 (Pkd1) gene is required for the responses of osteochondroprogenitor cells to midpalatal suture expansion in mice. Bone (2009) 0.96
A novel mutation causing nephronophthisis in the Lewis polycystic kidney rat localises to a conserved RCC1 domain in Nek8. BMC Genomics (2012) 0.96
Polycystin-1 surface localization is stimulated by polycystin-2 and cleavage at the G protein-coupled receptor proteolytic site. Mol Biol Cell (2010) 0.96
Primary cilia: highly sophisticated biological sensors. Sensors (Basel) (2009) 0.95
Pkd1 and Pkd2 are required for normal placental development. PLoS One (2010) 0.94
Hyperphosphorylation of polycystin-2 at a critical residue in disease reveals an essential role for polycystin-1-regulated dephosphorylation. Hum Mol Genet (2013) 0.94
Loss of polycystin-1 or polycystin-2 results in dysregulated apolipoprotein expression in murine tissues via alterations in nuclear hormone receptors. Hum Mol Genet (2005) 0.94
Novel targets for the treatment of autosomal dominant polycystic kidney disease. Expert Opin Investig Drugs (2010) 0.94
Polycystin-1 C-terminal cleavage is modulated by polycystin-2 expression. J Biol Chem (2009) 0.93
Cyst formation in the PKD2 (1-703) transgenic rat precedes deregulation of proliferation-related pathways. BMC Nephrol (2010) 0.93
Novel role for polycystin-1 in modulating cell proliferation through calcium oscillations in kidney cells. Cell Prolif (2008) 0.93
The γ-secretase cleavage product of polycystin-1 regulates TCF and CHOP-mediated transcriptional activation through a p300-dependent mechanism. Dev Cell (2011) 0.92
Regulation of STATs by polycystin-1 and their role in polycystic kidney disease. JAKSTAT (2013) 0.91
Acceleration of polycystic kidney disease progression in cpk mice carrying a deletion in the homeodomain protein Cux1. Am J Physiol Renal Physiol (2008) 0.91
Reduced Notch signaling leads to renal cysts and papillary microadenomas. J Am Soc Nephrol (2010) 0.91
CDK inhibitors R-roscovitine and S-CR8 effectively block renal and hepatic cystogenesis in an orthologous model of ADPKD. Cell Cycle (2012) 0.91
Drug discovery for polycystic kidney disease. Acta Pharmacol Sin (2011) 0.90
Polycystins and partners: proposed role in mechanosensitivity. J Physiol (2014) 0.89
The isolated polycystin-1 COOH-terminal can activate or block polycystin-1 signaling. Biochem Biophys Res Commun (2007) 0.88
Ouabain activates the Na-K-ATPase signalosome to induce autosomal dominant polycystic kidney disease cell proliferation. Am J Physiol Renal Physiol (2011) 0.87
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Fatty Acid Oxidation is Impaired in An Orthologous Mouse Model of Autosomal Dominant Polycystic Kidney Disease. EBioMedicine (2016) 0.86
Naturally occurring osmolytes modulate the nanomechanical properties of polycystic kidney disease domains. J Biol Chem (2010) 0.86
Cellular and molecular function of mucolipins (TRPML) and polycystin 2 (TRPP2). Pflugers Arch (2005) 0.85
Cilium, centrosome and cell cycle regulation in polycystic kidney disease. Biochim Biophys Acta (2011) 0.85
Transcriptional control of terminal nephron differentiation. Am J Physiol Renal Physiol (2008) 0.85
Polycystin-1 regulates amphiregulin expression through CREB and AP1 signalling: implications in ADPKD cell proliferation. J Mol Med (Berl) (2012) 0.85
A mechanistic approach to inherited polycystic kidney disease. Pediatr Nephrol (2005) 0.84
RIP-ed and ready to dance: new mechanisms for polycystin-1 signaling. J Clin Invest (2004) 0.84
Impaired formation of desmosomal junctions in ADPKD epithelia. Histochem Cell Biol (2005) 0.84
New insights into ciliary function: kidney cysts and photoreceptors. Proc Natl Acad Sci U S A (2003) 0.84
STAT1 is required for redifferentiation during Madin-Darby canine kidney tubulogenesis. Mol Biol Cell (2010) 0.83
Transgenic overexpression of Anks6(p.R823W) causes polycystic kidney disease in rats. Am J Pathol (2010) 0.83
Regulation of cell proliferation and differentiation in the kidney. Front Biosci (Landmark Ed) (2009) 0.82
Phosphoinositide 3-Kinase-C2α Regulates Polycystin-2 Ciliary Entry and Protects against Kidney Cyst Formation. J Am Soc Nephrol (2015) 0.81
A high-resolution structure of the EF-hand domain of human polycystin-2. Protein Sci (2014) 0.81
Kidney: polycystic kidney disease. Wiley Interdiscip Rev Dev Biol (2014) 0.81
The MicroArray Quality Control (MAQC) project shows inter- and intraplatform reproducibility of gene expression measurements. Nat Biotechnol (2006) 30.90
Intraepithelial CD8+ tumor-infiltrating lymphocytes and a high CD8+/regulatory T cell ratio are associated with favorable prognosis in ovarian cancer. Proc Natl Acad Sci U S A (2005) 10.89
The MicroArray Quality Control (MAQC)-II study of common practices for the development and validation of microarray-based predictive models. Nat Biotechnol (2010) 7.08
RNA interference screen for human genes associated with West Nile virus infection. Nature (2008) 4.93
The mTOR pathway is regulated by polycystin-1, and its inhibition reverses renal cystogenesis in polycystic kidney disease. Proc Natl Acad Sci U S A (2006) 4.56
A critical developmental switch defines the kinetics of kidney cyst formation after loss of Pkd1. Nat Med (2007) 4.40
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
The Surgical Mortality Probability Model: derivation and validation of a simple risk prediction rule for noncardiac surgery. Ann Surg (2012) 3.20
Tumor-infiltrating NY-ESO-1-specific CD8+ T cells are negatively regulated by LAG-3 and PD-1 in human ovarian cancer. Proc Natl Acad Sci U S A (2010) 3.12
Molecular characterization of the Arabidopsis 9-cis epoxycarotenoid dioxygenase gene family. Plant J (2003) 3.01
TRPP2 and TRPV4 form a polymodal sensory channel complex. J Cell Biol (2008) 2.88
Toll-like receptor 7 mitigates lethal West Nile encephalitis via interleukin 23-dependent immune cell infiltration and homing. Immunity (2009) 2.66
Age-associated decrease in TLR function in primary human dendritic cells predicts influenza vaccine response. J Immunol (2010) 2.63
Vaccination with an NY-ESO-1 peptide of HLA class I/II specificities induces integrated humoral and T cell responses in ovarian cancer. Proc Natl Acad Sci U S A (2007) 2.61
The balance of reproducibility, sensitivity, and specificity of lists of differentially expressed genes in microarray studies. BMC Bioinformatics (2008) 2.54
NY-ESO-1 and LAGE-1 cancer-testis antigens are potential targets for immunotherapy in epithelial ovarian cancer. Cancer Res (2003) 2.53
Cleavage of polycystin-1 requires the receptor for egg jelly domain and is disrupted by human autosomal-dominant polycystic kidney disease 1-associated mutations. Proc Natl Acad Sci U S A (2002) 2.29
A functional floxed allele of Pkd1 that can be conditionally inactivated in vivo. J Am Soc Nephrol (2004) 1.97
Phase II trial of 17-allylamino-17-demethoxygeldanamycin in patients with metastatic melanoma. Clin Cancer Res (2008) 1.94
Dysregulation of TLR3 impairs the innate immune response to West Nile virus in the elderly. J Virol (2008) 1.93
Chromatin-remodelling factor BRG1 selectively activates a subset of interferon-alpha-inducible genes. Nat Cell Biol (2002) 1.91
Detection of epithelial ovarian cancer using 1H-NMR-based metabonomics. Int J Cancer (2005) 1.84
A preparatively convenient ligand-free catalytic PEG 2000 Suzuki-Miyaura coupling. J Org Chem (2009) 1.79
Molecular advances in autosomal dominant polycystic kidney disease. Adv Chronic Kidney Dis (2010) 1.77
ArrayTrack: an FDA and public genomic tool. Methods Mol Biol (2009) 1.71
Rapamycin ameliorates PKD resulting from conditional inactivation of Pkd1. J Am Soc Nephrol (2010) 1.70
Spectrum of mutations in the gene for autosomal recessive polycystic kidney disease (ARPKD/PKHD1). J Am Soc Nephrol (2003) 1.70
T-cell factor/β-catenin activity is suppressed in two different models of autosomal dominant polycystic kidney disease. Kidney Int (2011) 1.69
Detection of B-RAF and N-RAS mutations in human melanoma. J Am Coll Surg (2005) 1.66
Polycystin 2 interacts with type I inositol 1,4,5-trisphosphate receptor to modulate intracellular Ca2+ signaling. J Biol Chem (2005) 1.64
Defective glucose metabolism in polycystic kidney disease identifies a new therapeutic strategy. Nat Med (2013) 1.62
Polycystin-1 regulates extracellular signal-regulated kinase-dependent phosphorylation of tuberin to control cell size through mTOR and its downstream effectors S6K and 4EBP1. Mol Cell Biol (2009) 1.58
Intragenic motifs regulate the transcriptional complexity of Pkhd1/PKHD1. J Mol Med (Berl) (2014) 1.56
Autosomal dominant polycystic kidney disease: molecular genetics and pathophysiology. J Lab Clin Med (2003) 1.56
The redox-sensitive cation channel TRPM2 modulates phagocyte ROS production and inflammation. Nat Immunol (2011) 1.55
Essential role of cleavage of Polycystin-1 at G protein-coupled receptor proteolytic site for kidney tubular structure. Proc Natl Acad Sci U S A (2007) 1.52
A novel mouse model reveals that polycystin-1 deficiency in ependyma and choroid plexus results in dysfunctional cilia and hydrocephalus. PLoS One (2009) 1.51
A Pkd1-Fbn1 genetic interaction implicates TGF-β signaling in the pathogenesis of vascular complications in autosomal dominant polycystic kidney disease. J Am Soc Nephrol (2013) 1.49
Polycystin-1 induces cell migration by regulating phosphatidylinositol 3-kinase-dependent cytoskeletal rearrangements and GSK3beta-dependent cell cell mechanical adhesion. Mol Biol Cell (2007) 1.47
Polyductin undergoes notch-like processing and regulated release from primary cilia. Hum Mol Genet (2007) 1.44
Genetic interaction studies link autosomal dominant and recessive polycystic kidney disease in a common pathway. Hum Mol Genet (2007) 1.43
Polyductin, the PKHD1 gene product, comprises isoforms expressed in plasma membrane, primary cilium, and cytoplasm. Kidney Int (2004) 1.42
Identification and characterization of Pkhd1, the mouse orthologue of the human ARPKD gene. J Am Soc Nephrol (2002) 1.41
Mobilization of endothelial progenitor cells from bone marrow is impaired in a piglet model of acute respiratory distress syndrome. Pediatr Crit Care Med (2013) 1.41
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PKHD1 mutations in autosomal recessive polycystic kidney disease (ARPKD). Hum Mutat (2004) 1.40
Linking cilia to Wnts. Nat Genet (2005) 1.40
Dysregulation of human Toll-like receptor function in aging. Ageing Res Rev (2010) 1.39
Embryonic arrest at midgestation and disruption of Notch signaling produced by the absence of both epsin 1 and epsin 2 in mice. Proc Natl Acad Sci U S A (2009) 1.38
Dynamin triple knockout cells reveal off target effects of commonly used dynamin inhibitors. J Cell Sci (2013) 1.38
A paradoxical role for neutrophils in the pathogenesis of West Nile virus. J Infect Dis (2010) 1.37
Rescue screens with secreted proteins reveal compensatory potential of receptor tyrosine kinases in driving cancer growth. Cancer Discov (2012) 1.37
Resveratrol protects human lens epithelial cells against H2O2-induced oxidative stress by increasing catalase, SOD-1, and HO-1 expression. Mol Vis (2010) 1.35
IL-10 signaling blockade controls murine West Nile virus infection. PLoS Pathog (2009) 1.35
Role of polycystins in renal tubulogenesis. Trends Cell Biol (2003) 1.35
Loss of mouse Ikbkap, a subunit of elongator, leads to transcriptional deficits and embryonic lethality that can be rescued by human IKBKAP. Mol Cell Biol (2008) 1.35
Fusion of two malaria vaccine candidate antigens enhances product yield, immunogenicity, and antibody-mediated inhibition of parasite growth in vitro. J Immunol (2004) 1.33
Loss of Bardet Biedl syndrome proteins causes defects in peripheral sensory innervation and function. Proc Natl Acad Sci U S A (2007) 1.32
Evaluating the clinical utility of a molecular genetic test for polycystic kidney disease. Mol Genet Metab (2007) 1.31
Characterization of cis-autoproteolysis of polycystin-1, the product of human polycystic kidney disease 1 gene. J Biol Chem (2007) 1.26
Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis: summary statement of a first National Institutes of Health/Office of Rare Diseases conference. J Pediatr (2006) 1.26
Soluble epoxide hydrolase inhibition and gene deletion are protective against myocardial ischemia-reperfusion injury in vivo. Am J Physiol Heart Circ Physiol (2008) 1.25
Versatile peroxidase oxidation of high redox potential aromatic compounds: site-directed mutagenesis, spectroscopic and crystallographic investigation of three long-range electron transfer pathways. J Mol Biol (2005) 1.24
Polycystin-1 induces resistance to apoptosis through the phosphatidylinositol 3-kinase/Akt signaling pathway. J Am Soc Nephrol (2006) 1.23
High density lipoprotein structure. Front Biosci (2003) 1.22
A tryptophan neutral radical in the oxidized state of versatile peroxidase from Pleurotus eryngii: a combined multifrequency EPR and density functional theory study. J Biol Chem (2006) 1.20
In vitro anti-tumor activity of 2',4'-dihydroxy-6'-methoxy-3',5'-dimethylchalcone against six established human cancer cell lines. Pharmacol Res (2004) 1.19
Pkd1 haploinsufficiency increases renal damage and induces microcyst formation following ischemia/reperfusion. J Am Soc Nephrol (2009) 1.19
Therapeutic potential and mechanism of kinetin as a treatment for the human splicing disease familial dysautonomia. J Mol Med (Berl) (2007) 1.17
A C-type lectin collaborates with a CD45 phosphatase homolog to facilitate West Nile virus infection of mosquitoes. Cell (2010) 1.17
Polymerization of MIP-1 chemokine (CCL3 and CCL4) and clearance of MIP-1 by insulin-degrading enzyme. EMBO J (2010) 1.16
Comparison of outcomes for laparoscopically assisted and open radical distal gastrectomy with lymphadenectomy for advanced gastric cancer. Surg Endosc (2011) 1.15
Influence of ApoA-I structure on the ABCA1-mediated efflux of cellular lipids. J Biol Chem (2004) 1.15
miR-133b, a muscle-specific microRNA, is a novel prognostic marker that participates in the progression of human colorectal cancer via regulation of CXCR4 expression. Mol Cancer (2013) 1.15
Milder presentation of recessive polycystic kidney disease requires presence of amino acid substitution mutations. J Am Soc Nephrol (2003) 1.13
Prospects for mTOR inhibitor use in patients with polycystic kidney disease and hamartomatous diseases. Clin J Am Soc Nephrol (2010) 1.12
Notch-1 signaling facilitates survivin expression in human non-small cell lung cancer cells. Cancer Biol Ther (2011) 1.12
MiR-483-5p suppresses the proliferation of glioma cells via directly targeting ERK1. FEBS Lett (2012) 1.11
ASC/PYCARD and caspase-1 regulate the IL-18/IFN-gamma axis during Anaplasma phagocytophilum infection. J Immunol (2007) 1.10
Mypt1-mediated spatial positioning of Bmp2-producing cells is essential for liver organogenesis. Development (2008) 1.10
Influence of CD4+CD25+ regulatory T cells on low/high-avidity CD4+ T cells following peptide vaccination. J Immunol (2006) 1.10
Mold(2), molecular descriptors from 2D structures for chemoinformatics and toxicoinformatics. J Chem Inf Model (2008) 1.09
mTOR inhibitors in polycystic kidney disease. N Engl J Med (2010) 1.09