Published in J Pediatr on August 01, 2006
Clinical and Molecular Investigations Into Ciliopathies | NCT00068224
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Kinesin family member 12 is a candidate polycystic kidney disease modifier in the cpk mouse. J Am Soc Nephrol (2005) 1.06
Predominant extrahepatic biliary disease in autosomal recessive polycystic kidney disease: a new association. Pediatr Transplant (2006) 1.02
Haplotype analysis improves molecular diagnostics of autosomal recessive polycystic kidney disease. Am J Kidney Dis (2005) 1.01
Epidermal growth factor receptor tyrosine kinase inhibitors. Br J Cancer (2004) 0.98
Calcium signaling and polycystin-2. Biochem Biophys Res Commun (2004) 0.97
Expression of components of the renin-angiotensin system in autosomal recessive polycystic kidney disease. J Histochem Cytochem (2005) 0.93
Fibrocystin interacts with CAML, a protein involved in Ca2+ signaling. Biochem Biophys Res Commun (2005) 0.90
Cyst fluid composition in human autosomal recessive polycystic kidney disease. Pediatr Nephrol (2005) 0.89
New options for prenatal diagnosis in autosomal recessive polycystic kidney disease by mutation analysis of the PKHD1 gene. Clin Genet (2004) 0.83
Variable expression of autosomal recessive polycystic kidney disease and congenital hepatic fibrosis within a family. Am J Med Genet (1988) 0.81
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Emerging therapies for polycystic kidney disease. Curr Opin Pharmacol (2005) 0.79
Polycystins 1 and 2 mediate mechanosensation in the primary cilium of kidney cells. Nat Genet (2003) 13.33
New equations to estimate GFR in children with CKD. J Am Soc Nephrol (2009) 11.48
The genome of the diatom Thalassiosira pseudonana: ecology, evolution, and metabolism. Science (2004) 9.97
NT5E mutations and arterial calcifications. N Engl J Med (2011) 8.71
Competency-based medical education: theory to practice. Med Teach (2010) 7.76
Diffusion-weighted magnetic resonance imaging as a cancer biomarker: consensus and recommendations. Neoplasia (2009) 7.44
Volume progression in polycystic kidney disease. N Engl J Med (2006) 7.16
Risk HLA-DQA1 and PLA(2)R1 alleles in idiopathic membranous nephropathy. N Engl J Med (2011) 6.34
Pten regulates neuronal arborization and social interaction in mice. Neuron (2006) 6.11
Imaging of angiogenesis: from microscope to clinic. Nat Med (2003) 5.51
Phenotype and course of Hutchinson-Gilford progeria syndrome. N Engl J Med (2008) 5.34
Natural history of alkaptonuria. N Engl J Med (2002) 5.32
Blood pressure in early autosomal dominant polycystic kidney disease. N Engl J Med (2014) 5.01
Epilepsy, ataxia, sensorineural deafness, tubulopathy, and KCNJ10 mutations. N Engl J Med (2009) 4.96
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
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Rapid cancer detection by topically spraying a γ-glutamyltranspeptidase-activated fluorescent probe. Sci Transl Med (2011) 4.72
Early-onset stroke and vasculopathy associated with mutations in ADA2. N Engl J Med (2014) 4.70
Magnetic resonance imaging/ultrasound-fusion biopsy significantly upgrades prostate cancer versus systematic 12-core transrectal ultrasound biopsy. Eur Urol (2013) 4.64
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
The gene mutated in autosomal recessive polycystic kidney disease encodes a large, receptor-like protein. Nat Genet (2002) 4.54
Platelet polyphosphates are proinflammatory and procoagulant mediators in vivo. Cell (2009) 4.50
A critical developmental switch defines the kinetics of kidney cyst formation after loss of Pkd1. Nat Med (2007) 4.40
The intraflagellar transport protein IFT20 is associated with the Golgi complex and is required for cilia assembly. Mol Biol Cell (2006) 4.18
Clearance properties of nano-sized particles and molecules as imaging agents: considerations and caveats. Nanomedicine (Lond) (2008) 4.08
PDGFRalphaalpha signaling is regulated through the primary cilium in fibroblasts. Curr Biol (2005) 4.04
A genetic screen in zebrafish identifies cilia genes as a principal cause of cystic kidney. Development (2004) 3.97
A test of climate, sun, and culture relationships from an 1810-year Chinese cave record. Science (2008) 3.94
Genital transmission of HPV in a mouse model is potentiated by nonoxynol-9 and inhibited by carrageenan. Nat Med (2007) 3.88
Design and methods of the Chronic Kidney Disease in Children (CKiD) prospective cohort study. Clin J Am Soc Nephrol (2006) 3.84
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
A pilot study of pioglitazone treatment for nonalcoholic steatohepatitis. Hepatology (2004) 3.61
The tiny eukaryote Ostreococcus provides genomic insights into the paradox of plankton speciation. Proc Natl Acad Sci U S A (2007) 3.58
Polycystin-2 localizes to kidney cilia and the ciliary level is elevated in orpk mice with polycystic kidney disease. Curr Biol (2002) 3.55
Randomized clinical trial of long-acting somatostatin for autosomal dominant polycystic kidney and liver disease. J Am Soc Nephrol (2010) 3.47
Genetic and functional studies implicate HIF1α as a 14q kidney cancer suppressor gene. Cancer Discov (2011) 3.45
New strategies for fluorescent probe design in medical diagnostic imaging. Chem Rev (2010) 3.35
Endothelial cilia are fluid shear sensors that regulate calcium signaling and nitric oxide production through polycystin-1. Circulation (2008) 3.33
Comprehensive molecular diagnostics in autosomal dominant polycystic kidney disease. J Am Soc Nephrol (2007) 3.26
Germline BHD-mutation spectrum and phenotype analysis of a large cohort of families with Birt-Hogg-Dubé syndrome. Am J Hum Genet (2005) 3.26
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Large-scale proteomics and phosphoproteomics of urinary exosomes. J Am Soc Nephrol (2008) 3.22
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
Cell signalling by microRNA165/6 directs gene dose-dependent root cell fate. Nature (2010) 3.19
The transmembrane protein meckelin (MKS3) is mutated in Meckel-Gruber syndrome and the wpk rat. Nat Genet (2006) 3.19
Actomyosin-mediated cellular tension drives increased tissue stiffness and β-catenin activation to induce epidermal hyperplasia and tumor growth. Cancer Cell (2011) 3.19
Selective molecular imaging of viable cancer cells with pH-activatable fluorescence probes. Nat Med (2008) 3.15
Prostate cancer: value of multiparametric MR imaging at 3 T for detection--histopathologic correlation. Radiology (2010) 3.12
PKD1 induces p21(waf1) and regulation of the cell cycle via direct activation of the JAK-STAT signaling pathway in a process requiring PKD2. Cell (2002) 3.09
Functional studies of the kidney of living animals using multicolor two-photon microscopy. Am J Physiol Cell Physiol (2002) 3.09
Research capacity. Enabling the genomic revolution in Africa. Science (2014) 3.05
Acute liver failure in children: the first 348 patients in the pediatric acute liver failure study group. J Pediatr (2006) 3.03
Incompletely penetrant PKD1 alleles suggest a role for gene dosage in cyst initiation in polycystic kidney disease. Kidney Int (2009) 2.99
Intraflagellar transport is required for polarized recycling of the TCR/CD3 complex to the immune synapse. Nat Cell Biol (2009) 2.94
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
TRPP2 and TRPV4 form a polymodal sensory channel complex. J Cell Biol (2008) 2.88
Circulating suPAR in two cohorts of primary FSGS. J Am Soc Nephrol (2012) 2.83
Disorders of lysosome-related organelle biogenesis: clinical and molecular genetics. Annu Rev Genomics Hum Genet (2008) 2.83
Imaging prostate cancer: a multidisciplinary perspective. Radiology (2007) 2.79
Kidney volume and functional outcomes in autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol (2012) 2.78
Circulating fibrocytes as biomarker of prognosis in Hermansky-Pudlak syndrome. Am J Respir Crit Care Med (2014) 2.77
Interleukin-17 and interferon-gamma are produced concomitantly by human coronary artery-infiltrating T cells and act synergistically on vascular smooth muscle cells. Circulation (2009) 2.77
Cancer cell-selective in vivo near infrared photoimmunotherapy targeting specific membrane molecules. Nat Med (2011) 2.76
Vasopressin directly regulates cyst growth in polycystic kidney disease. J Am Soc Nephrol (2007) 2.71
Ascorbate in pharmacologic concentrations selectively generates ascorbate radical and hydrogen peroxide in extracellular fluid in vivo. Proc Natl Acad Sci U S A (2007) 2.71
Loss of cilia suppresses cyst growth in genetic models of autosomal dominant polycystic kidney disease. Nat Genet (2013) 2.71
Mouse Rev1 protein interacts with multiple DNA polymerases involved in translesion DNA synthesis. EMBO J (2003) 2.69
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
Vascular pathology of medial arterial calcifications in NT5E deficiency: implications for the role of adenosine in pseudoxanthoma elasticum. Mol Genet Metab (2011) 2.65
Neutropenia during combination therapy of interferon alfa and ribavirin for chronic hepatitis C. Hepatology (2002) 2.64
Akt-RSK-S6 kinase signaling networks activated by oncogenic receptor tyrosine kinases. Sci Signal (2010) 2.64
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Experimental model for paroxysmal atrial fibrillation arising at the pulmonary vein-atrial junctions. Heart Rhythm (2006) 2.63
Utility of multiparametric magnetic resonance imaging suspicion levels for detecting prostate cancer. J Urol (2013) 2.62
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Nomograms for predicting annual resolution rate of primary vesicoureteral reflux: results from 2,462 children. J Urol (2009) 2.59
The Chlamydomonas reinhardtii BBSome is an IFT cargo required for export of specific signaling proteins from flagella. J Cell Biol (2009) 2.55
Cellular and subcellular localization of the ARPKD protein; fibrocystin is expressed on primary cilia. Hum Mol Genet (2003) 2.55
Correlation of magnetic resonance imaging tumor volume with histopathology. J Urol (2012) 2.55
In vivo molecular imaging of cancer with a quenching near-infrared fluorescent probe using conjugates of monoclonal antibodies and indocyanine green. Cancer Res (2009) 2.51
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Coupling of human immunodeficiency virus type 1 fusion to virion maturation: a novel role of the gp41 cytoplasmic tail. J Virol (2004) 2.47
Simultaneous multicolor imaging of five different lymphatic basins using quantum dots. Nano Lett (2007) 2.46
Cholangiocyte cilia detect changes in luminal fluid flow and transmit them into intracellular Ca2+ and cAMP signaling. Gastroenterology (2006) 2.43
11C-Acetate PET/CT in localized prostate cancer: a study with MRI and histopathologic correlation. J Nucl Med (2012) 2.42
Natural killer cells are polarized toward cytotoxicity in chronic hepatitis C in an interferon-alfa-dependent manner. Gastroenterology (2009) 2.41
Host response to translocated microbial products predicts outcomes of patients with HBV or HCV infection. Gastroenterology (2011) 2.39
Is apparent diffusion coefficient associated with clinical risk scores for prostate cancers that are visible on 3-T MR images? Radiology (2010) 2.38