Published in Gastroenterology on November 01, 2003
Polycystic kidney disease. Annu Rev Med (2009) 4.78
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Fibrocystin/polyductin modulates renal tubular formation by regulating polycystin-2 expression and function. J Am Soc Nephrol (2008) 2.04
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Cholangiocyte cilia express TRPV4 and detect changes in luminal tonicity inducing bicarbonate secretion. Proc Natl Acad Sci U S A (2007) 1.72
Ciliary and centrosomal defects associated with mutation and depletion of the Meckel syndrome genes MKS1 and MKS3. Hum Mol Genet (2009) 1.61
The cAMP effectors Epac and protein kinase a (PKA) are involved in the hepatic cystogenesis of an animal model of autosomal recessive polycystic kidney disease (ARPKD). Hepatology (2009) 1.58
Cholangiocyte primary cilia are chemosensory organelles that detect biliary nucleotides via P2Y12 purinergic receptors. Am J Physiol Gastrointest Liver Physiol (2008) 1.56
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PKHD1 protein encoded by the gene for autosomal recessive polycystic kidney disease associates with basal bodies and primary cilia in renal epithelial cells. Proc Natl Acad Sci U S A (2004) 1.42
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Polycystic kidney disease protein fibrocystin localizes to the mitotic spindle and regulates spindle bipolarity. Hum Mol Genet (2010) 0.95
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HDAC6 is overexpressed in cystic cholangiocytes and its inhibition reduces cystogenesis. Am J Pathol (2014) 0.78
Safety study of somatostatin analogue octreotide for autosomal dominant polycystic kidney disease in Japan. Clin Exp Nephrol (2014) 0.78
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TGR5 contributes to hepatic cystogenesis in rodents with polycystic liver diseases via cAMP/Gαs signaling. Hepatology (2017) 0.75
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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
Polycystic kidney disease. Annu Rev Med (2009) 4.78
The gene mutated in autosomal recessive polycystic kidney disease encodes a large, receptor-like protein. Nat Genet (2002) 4.54
Inhibition of renal cystic disease development and progression by a vasopressin V2 receptor antagonist. Nat Med (2003) 3.78
Strategies for improving attendance at medical grand rounds at an academic medical center. Mayo Clin Proc (2003) 3.71
Autosomal dominant polycystic kidney disease: the last 3 years. Kidney Int (2009) 3.66
Randomized clinical trial of long-acting somatostatin for autosomal dominant polycystic kidney and liver disease. J Am Soc Nephrol (2010) 3.47
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Cryptosporidiosis. N Engl J Med (2002) 3.24
The transmembrane protein meckelin (MKS3) is mutated in Meckel-Gruber syndrome and the wpk rat. Nat Genet (2006) 3.19
Mutations in DNMT1 cause hereditary sensory neuropathy with dementia and hearing loss. Nat Genet (2011) 3.16
Effective treatment of an orthologous model of autosomal dominant polycystic kidney disease. Nat Med (2004) 3.09
Incompletely penetrant PKD1 alleles suggest a role for gene dosage in cyst initiation in polycystic kidney disease. Kidney Int (2009) 2.99
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Effectiveness of vasopressin V2 receptor antagonists OPC-31260 and OPC-41061 on polycystic kidney disease development in the PCK rat. J Am Soc Nephrol (2005) 2.92
A cellular micro-RNA, let-7i, regulates Toll-like receptor 4 expression and contributes to cholangiocyte immune responses against Cryptosporidium parvum infection. J Biol Chem (2007) 2.89
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Vasopressin directly regulates cyst growth in polycystic kidney disease. J Am Soc Nephrol (2007) 2.71
Octreotide inhibits hepatic cystogenesis in a rodent model of polycystic liver disease by reducing cholangiocyte adenosine 3',5'-cyclic monophosphate. Gastroenterology (2006) 2.68
Volume progression in autosomal dominant polycystic kidney disease: the major factor determining clinical outcomes. Clin J Am Soc Nephrol (2005) 2.66
Cellular and subcellular localization of the ARPKD protein; fibrocystin is expressed on primary cilia. Hum Mol Genet (2003) 2.55
Lowering of Pkd1 expression is sufficient to cause polycystic kidney disease. Hum Mol Genet (2004) 2.47
Cholangiocyte cilia detect changes in luminal fluid flow and transmit them into intracellular Ca2+ and cAMP signaling. Gastroenterology (2006) 2.43
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
Clinical and molecular characterization defines a broadened spectrum of autosomal recessive polycystic kidney disease (ARPKD). Medicine (Baltimore) (2006) 2.34
Sirolimus reduces polycystic liver volume in ADPKD patients. J Am Soc Nephrol (2008) 2.26
Polycystic liver disease: a critical appraisal of hepatic resection, cyst fenestration, and liver transplantation. Ann Surg (2009) 2.23
MicroRNA-21 is overexpressed in human cholangiocarcinoma and regulates programmed cell death 4 and tissue inhibitor of metalloproteinase 3. Hepatology (2009) 2.23
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
Characterization of PKD protein-positive exosome-like vesicles. J Am Soc Nephrol (2009) 2.13
Molecular pathogenesis of ADPKD: the polycystin complex gets complex. Kidney Int (2005) 2.13
Perceived impact of duty hours limits on the fragmentation of patient care: results from an academic health center. Am J Med (2005) 2.10
Mutations in SEC63 cause autosomal dominant polycystic liver disease. Nat Genet (2004) 1.96
Loss of polycystin-1 in human cyst-lining epithelia leads to ciliary dysfunction. J Am Soc Nephrol (2006) 1.95
Isolation and characterization of cholangiocyte primary cilia. Am J Physiol Gastrointest Liver Physiol (2006) 1.92
Multiple TLRs are expressed in human cholangiocytes and mediate host epithelial defense responses to Cryptosporidium parvum via activation of NF-kappaB. J Immunol (2005) 1.88
Biliary exosomes influence cholangiocyte regulatory mechanisms and proliferation through interaction with primary cilia. Am J Physiol Gastrointest Liver Physiol (2010) 1.85
MicroRNA-513 regulates B7-H1 translation and is involved in IFN-gamma-induced B7-H1 expression in cholangiocytes. J Immunol (2009) 1.76
Functional polycystin-1 dosage governs autosomal dominant polycystic kidney disease severity. J Clin Invest (2012) 1.74
Cyclic AMP regulates bicarbonate secretion in cholangiocytes through release of ATP into bile. Gastroenterology (2007) 1.73
Cholangiocyte cilia express TRPV4 and detect changes in luminal tonicity inducing bicarbonate secretion. Proc Natl Acad Sci U S A (2007) 1.72
Human ADPKD primary cyst epithelial cells with a novel, single codon deletion in the PKD1 gene exhibit defective ciliary polycystin localization and loss of flow-induced Ca2+ signaling. Am J Physiol Renal Physiol (2006) 1.72
Follow-up of intracranial aneurysms in autosomal-dominant polycystic kidney disease. Kidney Int (2004) 1.71
Young women with polycystic liver disease respond best to somatostatin analogues: a pooled analysis of individual patient data. Gastroenterology (2013) 1.66
A complete mutation screen of the ADPKD genes by DHPLC. Kidney Int (2002) 1.66
Equations to estimate creatinine excretion rate: the CKD epidemiology collaboration. Clin J Am Soc Nephrol (2010) 1.65
Angiotensin blockade in late autosomal dominant polycystic kidney disease. N Engl J Med (2014) 1.64
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Tolvaptan in autosomal dominant polycystic kidney disease: three years' experience. Clin J Am Soc Nephrol (2011) 1.63
Quantitative assessment of hepatic fibrosis in an animal model with magnetic resonance elastography. Magn Reson Med (2007) 1.61
Ciliary and centrosomal defects associated with mutation and depletion of the Meckel syndrome genes MKS1 and MKS3. Hum Mol Genet (2009) 1.61