Published in Am J Physiol Renal Physiol on April 01, 2002
TRPV4 dysfunction promotes renal cystogenesis in autosomal recessive polycystic kidney disease. J Am Soc Nephrol (2013) 1.60
Physiology and pathophysiology of the vasopressin-regulated renal water reabsorption. Pflugers Arch (2008) 1.37
Attenuated, flow-induced ATP release contributes to absence of flow-sensitive, purinergic Cai2+ signaling in human ADPKD cyst epithelial cells. Am J Physiol Renal Physiol (2009) 1.28
Loss of apical monocilia on collecting duct principal cells impairs ATP secretion across the apical cell surface and ATP-dependent and flow-induced calcium signals. Purinergic Signal (2007) 1.27
Polycystins and renovascular mechanosensory transduction. Nat Rev Nephrol (2010) 1.13
Regulation of the epithelial sodium channel by phosphatidylinositides: experiments, implications, and speculations. Pflugers Arch (2007) 1.10
ATP-2 interacts with the PLAT domain of LOV-1 and is involved in Caenorhabditis elegans polycystin signaling. Mol Biol Cell (2004) 1.02
Intrarenal localization of the plasma membrane ATP channel pannexin1. Am J Physiol Renal Physiol (2012) 0.99
Recent patents on novel P2X(7) receptor antagonists and their potential for reducing central nervous system inflammation. Recent Pat CNS Drug Discov (2010) 0.97
Purinergic signaling in the lumen of a normal nephron and in remodeled PKD encapsulated cysts. Purinergic Signal (2008) 0.97
ENaC, renal sodium excretion and extracellular ATP. Purinergic Signal (2009) 0.95
Novel targets for the treatment of autosomal dominant polycystic kidney disease. Expert Opin Investig Drugs (2010) 0.94
Introduction and perspective, historical note. Front Cell Neurosci (2013) 0.93
Coupled ATP and potassium efflux from intercalated cells. Am J Physiol Renal Physiol (2011) 0.92
Pentosan polysulfate treatment preserves renal autoregulation in ANG II-infused hypertensive rats via normalization of P2X1 receptor activation. Am J Physiol Renal Physiol (2010) 0.89
Extracellular ATP and zinc are co-secreted with insulin and activate multiple P2X purinergic receptor channels expressed by islet beta-cells to potentiate insulin secretion. Purinergic Signal (2008) 0.87
Hypoxia-inducible factor-1α causes renal cyst expansion through calcium-activated chloride secretion. J Am Soc Nephrol (2013) 0.87
Purinergic signalling in the kidney in health and disease. Purinergic Signal (2013) 0.84
Prostaglandin E(2) mediates proliferation and chloride secretion in ADPKD cystic renal epithelia. Am J Physiol Renal Physiol (2012) 0.83
P2 receptors in renal pathophysiology. Purinergic Signal (2009) 0.81
The dark side of extracellular ATP in kidney diseases. J Am Soc Nephrol (2014) 0.80
Deciphering physiological role of the mechanosensitive TRPV4 channel in the distal nephron. Am J Physiol Renal Physiol (2014) 0.80
Endogenous ATP release inhibits electrogenic Na⁺ absorption and stimulates Cl⁻ secretion in MDCK cells. Purinergic Signal (2007) 0.80
Purinergic signaling microenvironments: An introduction. Purinergic Signal (2008) 0.79
Impaired epithelial Na+ channel activity contributes to cystogenesis and development of autosomal recessive polycystic kidney disease in PCK rats. Pediatr Res (2014) 0.79
Role of extracellular ATP and P2 receptor signaling in regulating renal cyst growth and interstitial inflammation in polycystic kidney disease. Front Physiol (2013) 0.79
Implementing Patch Clamp and Live Fluorescence Microscopy to Monitor Functional Properties of Freshly Isolated PKD Epithelium. J Vis Exp (2015) 0.79
Autoregulation in PC12 cells via P2Y receptors: Evidence for non-exocytotic nucleotide release from neuroendocrine cells. Purinergic Signal (2007) 0.78
Cl- secretion in ATP-treated renal epithelial C7-MDCK cells is mediated by activation of P 2Y1 receptors, phospholipase A2 and protein kinase A. J Physiol (2005) 0.78
Purinergic inhibition of Na⁺,K⁺,Cl⁻ cotransport in C11-MDCK cells: Role of stress-activated protein kinases. Purinergic Signal (2007) 0.78
Functional and therapeutic importance of purinergic signaling in polycystic kidney disease. Am J Physiol Renal Physiol (2016) 0.75
Tolvaptan in patients with autosomal dominant polycystic kidney disease. N Engl J Med (2012) 9.88
Volume progression in polycystic kidney disease. N Engl J Med (2006) 7.16
Comparative genomics identifies a flagellar and basal body proteome that includes the BBS5 human disease gene. Cell (2004) 6.10
The polycystic kidney disease proteins, polycystin-1, polycystin-2, polaris, and cystin, are co-localized in renal cilia. J Am Soc Nephrol (2002) 5.58
Blood pressure in early autosomal dominant polycystic kidney disease. N Engl J Med (2014) 5.01
Renal structure in early autosomal-dominant polycystic kidney disease (ADPKD): The Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease (CRISP) cohort. Kidney Int (2003) 4.84
Candidate exome capture identifies mutation of SDCCAG8 as the cause of a retinal-renal ciliopathy. Nat Genet (2010) 3.99
Fibrosis--a common pathway to organ injury and failure. N Engl J Med (2015) 3.94
Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator. EMBO J (2003) 3.35
Comprehensive molecular diagnostics in autosomal dominant polycystic kidney disease. J Am Soc Nephrol (2007) 3.26
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
Identification of a urate transporter, ABCG2, with a common functional polymorphism causing gout. Proc Natl Acad Sci U S A (2009) 3.13
Angiotensin II directly stimulates ENaC activity in the cortical collecting duct via AT(1) receptors. J Am Soc Nephrol (2002) 3.04
Defective lipoxin-mediated anti-inflammatory activity in the cystic fibrosis airway. Nat Immunol (2004) 2.86
Kidney volume and functional outcomes in autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol (2012) 2.78
Increased water intake decreases progression of polycystic kidney disease in the PCK rat. J Am Soc Nephrol (2006) 2.77
Impact of the deltaF508 mutation in first nucleotide-binding domain of human cystic fibrosis transmembrane conductance regulator on domain folding and structure. J Biol Chem (2004) 2.66
Cyclic AMP promotes growth and secretion in human polycystic kidney epithelial cells. Kidney Int (2004) 2.55
Evaluation of urine biomarkers of kidney injury in polycystic kidney disease. Kidney Int (2012) 2.39
Magnetic resonance imaging evaluation of hepatic cysts in early autosomal-dominant polycystic kidney disease: the Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease cohort. Clin J Am Soc Nephrol (2005) 2.37
Cyclic AMP activates B-Raf and ERK in cyst epithelial cells from autosomal-dominant polycystic kidneys. Kidney Int (2003) 2.32
The absence of intrarenal ACE protects against hypertension. J Clin Invest (2013) 2.31
Calcium restriction allows cAMP activation of the B-Raf/ERK pathway, switching cells to a cAMP-dependent growth-stimulated phenotype. J Biol Chem (2004) 2.26
Cyst number but not the rate of cystic growth is associated with the mutated gene in autosomal dominant polycystic kidney disease. J Am Soc Nephrol (2006) 2.21
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
Cystin, a novel cilia-associated protein, is disrupted in the cpk mouse model of polycystic kidney disease. J Clin Invest (2002) 2.07
High citrate diet delays progression of renal insufficiency in the ClC-5 knockout mouse model of Dent's disease. Kidney Int (2005) 2.02
A Golgi-associated PDZ domain protein modulates cystic fibrosis transmembrane regulator plasma membrane expression. J Biol Chem (2001) 1.97
WNK4 enhances the degradation of NCC through a sortilin-mediated lysosomal pathway. J Am Soc Nephrol (2009) 1.87
Calcium restores a normal proliferation phenotype in human polycystic kidney disease epithelial cells. J Am Soc Nephrol (2005) 1.87
Loss of chloride channel ClC-5 impairs endocytosis by defective trafficking of megalin and cubilin in kidney proximal tubules. Proc Natl Acad Sci U S A (2003) 1.87
Macula densa cell signaling involves ATP release through a maxi anion channel. Proc Natl Acad Sci U S A (2003) 1.85
A classification of ductal plate malformations based on distinct pathogenic mechanisms of biliary dysmorphogenesis. Hepatology (2011) 1.84
Quantitative imaging of basic functions in renal (patho)physiology. Am J Physiol Renal Physiol (2006) 1.83
Killing of Klebsiella pneumoniae by human alveolar macrophages. Am J Physiol Lung Cell Mol Physiol (2002) 1.83
KCa3.1 potassium channels are critical for cAMP-dependent chloride secretion and cyst growth in autosomal-dominant polycystic kidney disease. Kidney Int (2008) 1.80
Cystin localizes to primary cilia via membrane microdomains and a targeting motif. J Am Soc Nephrol (2009) 1.79
Heightened epithelial Na+ channel-mediated Na+ absorption in a murine polycystic kidney disease model epithelium lacking apical monocilia. Am J Physiol Cell Physiol (2005) 1.74
PDZ domain interaction controls the endocytic recycling of the cystic fibrosis transmembrane conductance regulator. J Biol Chem (2002) 1.65
Angiotensin blockade in late autosomal dominant polycystic kidney disease. N Engl J Med (2014) 1.64
Polycystin 2 interacts with type I inositol 1,4,5-trisphosphate receptor to modulate intracellular Ca2+ signaling. J Biol Chem (2005) 1.64
Tolvaptan in autosomal dominant polycystic kidney disease: three years' experience. Clin J Am Soc Nephrol (2011) 1.63
Imaging for the prognosis of autosomal dominant polycystic kidney disease. Nat Rev Nephrol (2010) 1.63
Characterization of large rearrangements in autosomal dominant polycystic kidney disease and the PKD1/TSC2 contiguous gene syndrome. Kidney Int (2008) 1.58
Sorafenib inhibits cAMP-dependent ERK activation, cell proliferation, and in vitro cyst growth of human ADPKD cyst epithelial cells. Am J Physiol Renal Physiol (2010) 1.58
Intragenic motifs regulate the transcriptional complexity of Pkhd1/PKHD1. J Mol Med (Berl) (2014) 1.56
The medicinal use of water in renal disease. Kidney Int (2013) 1.56
Neuronal nitric oxide synthase: its role and regulation in macula densa cells. J Am Soc Nephrol (2003) 1.54
Magnetic resonance measurements of renal blood flow and disease progression in autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol (2006) 1.53
Purinergic receptor signaling at the basolateral membrane of macula densa cells. J Am Soc Nephrol (2002) 1.52
Drosophila TRPA1 channel mediates chemical avoidance in gustatory receptor neurons. Proc Natl Acad Sci U S A (2010) 1.51
Macrophage migration inhibitory factor promotes cyst growth in polycystic kidney disease. J Clin Invest (2015) 1.51
Novel approach to estimate kidney and cyst volumes using mid-slice magnetic resonance images in polycystic kidney disease. Am J Nephrol (2013) 1.50
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
Luminal NaCl delivery regulates basolateral PGE2 release from macula densa cells. J Clin Invest (2003) 1.49
Sonographic assessment of the severity and progression of autosomal dominant polycystic kidney disease: the Consortium of Renal Imaging Studies in Polycystic Kidney Disease (CRISP). Am J Kidney Dis (2005) 1.49
Multiphoton imaging of the glomerular permeability of angiotensinogen. J Am Soc Nephrol (2012) 1.48
N-methyl-D-aspartate receptor subunit NR3a expression and function in principal cells of the collecting duct. Am J Physiol Renal Physiol (2011) 1.48
Loss of primary cilia results in deregulated and unabated apical calcium entry in ARPKD collecting duct cells. Am J Physiol Renal Physiol (2006) 1.47
Urinary excretion of monocyte chemoattractant protein-1 in autosomal dominant polycystic kidney disease. J Am Soc Nephrol (2003) 1.46
Magnetic resonance measurements of renal blood flow as a marker of disease severity in autosomal-dominant polycystic kidney disease. Kidney Int (2003) 1.45
Polyductin undergoes notch-like processing and regulated release from primary cilia. Hum Mol Genet (2007) 1.44
Loss of the endothelial glycocalyx links albuminuria and vascular dysfunction. J Am Soc Nephrol (2012) 1.44
Impaired acidification in early endosomes of ClC-5 deficient proximal tubule. Biochem Biophys Res Commun (2005) 1.44
Genetic interaction studies link autosomal dominant and recessive polycystic kidney disease in a common pathway. Hum Mol Genet (2007) 1.43
Renal activation of extracellular signal-regulated kinase in rats with autosomal-dominant polycystic kidney disease. Kidney Int (2003) 1.42
Polyductin, the PKHD1 gene product, comprises isoforms expressed in plasma membrane, primary cilium, and cytoplasm. Kidney Int (2004) 1.42
A phase II, double-blind, randomized, placebo-controlled clinical trial of tgAAVCF using maxillary sinus delivery in patients with cystic fibrosis with antrostomies. Hum Gene Ther (2002) 1.40
Tolvaptan inhibits ERK-dependent cell proliferation, Cl⁻ secretion, and in vitro cyst growth of human ADPKD cells stimulated by vasopressin. Am J Physiol Renal Physiol (2011) 1.40
Rationale and design of the TEMPO (Tolvaptan Efficacy and Safety in Management of Autosomal Dominant Polycystic Kidney Disease and its Outcomes) 3-4 Study. Am J Kidney Dis (2011) 1.39
Respiratory epithelial gene expression in patients with mild and severe cystic fibrosis lung disease. Am J Respir Cell Mol Biol (2006) 1.39
A tumor necrosis factor-alpha-mediated pathway promoting autosomal dominant polycystic kidney disease. Nat Med (2008) 1.38
Comparison of methods for determining renal function decline in early autosomal dominant polycystic kidney disease: the consortium of radiologic imaging studies of polycystic kidney disease cohort. J Am Soc Nephrol (2006) 1.37
Phase I trial of intranasal and endobronchial administration of a recombinant adeno-associated virus serotype 2 (rAAV2)-CFTR vector in adult cystic fibrosis patients: a two-part clinical study. Hum Gene Ther (2003) 1.36
Drosophila TRPA1 channel is required to avoid the naturally occurring insect repellent citronellal. Curr Biol (2010) 1.36
Positional cloning of jcpk/bpk locus of the mouse. Mamm Genome (2003) 1.35
Characterization of aquaporin-6 as a nitrate channel in mammalian cells. Requirement of pore-lining residue threonine 63. J Biol Chem (2002) 1.35
A case for water in the treatment of polycystic kidney disease. Clin J Am Soc Nephrol (2009) 1.31
Sepsis biomarkers in unselected patients on admission to intensive or high-dependency care. Crit Care (2013) 1.29
Loss of apical monocilia on collecting duct principal cells impairs ATP secretion across the apical cell surface and ATP-dependent and flow-induced calcium signals. Purinergic Signal (2007) 1.27
Structures of a minimal human CFTR first nucleotide-binding domain as a monomer, head-to-tail homodimer, and pathogenic mutant. Protein Eng Des Sel (2010) 1.26
Polycystin-1 activates the calcineurin/NFAT (nuclear factor of activated T-cells) signaling pathway. J Biol Chem (2004) 1.26