PubRank

Peter C Harris

Author PubWeight™ 161.00‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Autosomal dominant polycystic kidney disease. Lancet 2007 8.55
2 Volume progression in polycystic kidney disease. N Engl J Med 2006 7.16
3 Progress and prospects in rat genetics: a community view. Nat Genet 2008 6.01
4 Blood pressure in early autosomal dominant polycystic kidney disease. N Engl J Med 2014 5.01
5 The gene mutated in autosomal recessive polycystic kidney disease encodes a large, receptor-like protein. Nat Genet 2002 4.54
6 Inhibition of renal cystic disease development and progression by a vasopressin V2 receptor antagonist. Nat Med 2003 3.78
7 Autosomal dominant polycystic kidney disease: the last 3 years. Kidney Int 2009 3.66
8 Randomized clinical trial of long-acting somatostatin for autosomal dominant polycystic kidney and liver disease. J Am Soc Nephrol 2010 3.47
9 Comprehensive molecular diagnostics in autosomal dominant polycystic kidney disease. J Am Soc Nephrol 2007 3.26
10 The transmembrane protein meckelin (MKS3) is mutated in Meckel-Gruber syndrome and the wpk rat. Nat Genet 2006 3.19
11 Effective treatment of an orthologous model of autosomal dominant polycystic kidney disease. Nat Med 2004 3.09
12 Incompletely penetrant PKD1 alleles suggest a role for gene dosage in cyst initiation in polycystic kidney disease. Kidney Int 2009 2.99
13 Mechanisms of Disease: autosomal dominant and recessive polycystic kidney diseases. Nat Clin Pract Nephrol 2006 2.95
14 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
15 Kidney volume and functional outcomes in autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol 2012 2.78
16 Vasopressin directly regulates cyst growth in polycystic kidney disease. J Am Soc Nephrol 2007 2.71
17 Octreotide inhibits hepatic cystogenesis in a rodent model of polycystic liver disease by reducing cholangiocyte adenosine 3',5'-cyclic monophosphate. Gastroenterology 2006 2.68
18 Cellular and subcellular localization of the ARPKD protein; fibrocystin is expressed on primary cilia. Hum Mol Genet 2003 2.55
19 Clinical and molecular characterization defines a broadened spectrum of autosomal recessive polycystic kidney disease (ARPKD). Medicine (Baltimore) 2006 2.34
20 Characterization of PKD protein-positive exosome-like vesicles. J Am Soc Nephrol 2009 2.13
21 Molecular pathogenesis of ADPKD: the polycystin complex gets complex. Kidney Int 2005 2.13
22 Loss of polycystin-1 in human cyst-lining epithelia leads to ciliary dysfunction. J Am Soc Nephrol 2006 1.95
23 Defects in cholangiocyte fibrocystin expression and ciliary structure in the PCK rat. Gastroenterology 2003 1.80
24 Functional polycystin-1 dosage governs autosomal dominant polycystic kidney disease severity. J Clin Invest 2012 1.74
25 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
26 Follow-up of intracranial aneurysms in autosomal-dominant polycystic kidney disease. Kidney Int 2004 1.71
27 A complete mutation screen of the ADPKD genes by DHPLC. Kidney Int 2002 1.66
28 Angiotensin blockade in late autosomal dominant polycystic kidney disease. N Engl J Med 2014 1.64
29 Molecular diagnostics of Meckel-Gruber syndrome highlights phenotypic differences between MKS1 and MKS3. Hum Genet 2007 1.64
30 Quantitative assessment of hepatic fibrosis in an animal model with magnetic resonance elastography. Magn Reson Med 2007 1.61
31 Ciliary and centrosomal defects associated with mutation and depletion of the Meckel syndrome genes MKS1 and MKS3. Hum Mol Genet 2009 1.61
32 The position of the polycystic kidney disease 1 (PKD1) gene mutation correlates with the severity of renal disease. J Am Soc Nephrol 2002 1.60
33 Genotype-phenotype correlations in autosomal dominant and autosomal recessive polycystic kidney disease. J Am Soc Nephrol 2007 1.60
34 Characterization of large rearrangements in autosomal dominant polycystic kidney disease and the PKD1/TSC2 contiguous gene syndrome. Kidney Int 2008 1.58
35 Identification of gene mutations in autosomal dominant polycystic kidney disease through targeted resequencing. J Am Soc Nephrol 2012 1.57
36 Identification, characterization, and localization of a novel kidney polycystin-1-polycystin-2 complex. J Biol Chem 2002 1.56
37 Incompletely penetrant PKD1 alleles mimic the renal manifestations of ARPKD. J Am Soc Nephrol 2010 1.52
38 The ENOS polymorphism is not associated with severity of renal disease in polycystic kidney disease 1. Am J Kidney Dis 2003 1.51
39 Biliary dysgenesis in the PCK rat, an orthologous model of autosomal recessive polycystic kidney disease. Am J Pathol 2004 1.49
40 Association of mutation position in polycystic kidney disease 1 (PKD1) gene and development of a vascular phenotype. Lancet 2003 1.47
41 B9D1 is revealed as a novel Meckel syndrome (MKS) gene by targeted exon-enriched next-generation sequencing and deletion analysis. Hum Mol Genet 2011 1.45
42 Pioglitazone improves the phenotype and molecular defects of a targeted Pkd1 mutant. Hum Mol Genet 2002 1.43
43 PKHD1 mutations in autosomal recessive polycystic kidney disease (ARPKD). Hum Mutat 2004 1.40
44 Comprehensive mutation screening in 55 probands with type 1 primary hyperoxaluria shows feasibility of a gene-based diagnosis. J Am Soc Nephrol 2007 1.39
45 A polycystin-1 multiprotein complex is disrupted in polycystic kidney disease cells. Mol Biol Cell 2004 1.39
46 Pkd2 haploinsufficiency alters intracellular calcium regulation in vascular smooth muscle cells. Hum Mol Genet 2003 1.39
47 Strategies targeting cAMP signaling in the treatment of polycystic kidney disease. J Am Soc Nephrol 2013 1.33
48 ANKS6 is a central component of a nephronophthisis module linking NEK8 to INVS and NPHP3. Nat Genet 2013 1.32
49 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
50 Analysis of the polycystins in aortic vascular smooth muscle cells. J Am Soc Nephrol 2003 1.27
51 Genetic mechanisms and signaling pathways in autosomal dominant polycystic kidney disease. J Clin Invest 2014 1.24
52 Hypercalcemia, hypercalciuria, and elevated calcitriol concentrations with autosomal dominant transmission due to CYP24A1 mutations: effects of ketoconazole therapy. J Clin Endocrinol Metab 2012 1.23
53 Proteolytic cleavage and nuclear translocation of fibrocystin is regulated by intracellular Ca2+ and activation of protein kinase C. J Biol Chem 2006 1.22
54 EGF receptor tyrosine kinase inhibition attenuates the development of PKD in Han:SPRD rats. Kidney Int 2003 1.22
55 A complete mutation screen of PKHD1 in autosomal-recessive polycystic kidney disease (ARPKD) pedigrees. Kidney Int 2003 1.19
56 Reduced ciliary polycystin-2 in induced pluripotent stem cells from polycystic kidney disease patients with PKD1 mutations. J Am Soc Nephrol 2013 1.14
57 Successful disease-specific induced pluripotent stem cell generation from patients with kidney transplantation. Stem Cell Res Ther 2011 1.12
58 Cyclic nucleotide signaling in polycystic kidney disease. Kidney Int 2009 1.09
59 Epitope-tagged Pkhd1 tracks the processing, secretion, and localization of fibrocystin. J Am Soc Nephrol 2011 1.09
60 Development of multiorgan pathology in the wpk rat model of polycystic kidney disease. Anat Rec A Discov Mol Cell Evol Biol 2004 1.05
61 Extended follow-up of unruptured intracranial aneurysms detected by presymptomatic screening in patients with autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol 2011 1.04
62 Pkd1 transgenic mice: adult model of polycystic kidney disease with extrarenal and renal phenotypes. Hum Mol Genet 2010 1.04
63 Autosomal dominant polycystic kidney disease coexisting with cystic fibrosis. J Nephrol 2006 1.03
64 Homophilic and heterophilic polycystin 1 interactions regulate E-cadherin recruitment and junction assembly in MDCK cells. J Cell Sci 2009 1.02
65 Haplotype analysis improves molecular diagnostics of autosomal recessive polycystic kidney disease. Am J Kidney Dis 2005 1.01
66 [Ca2+]i reduction increases cellular proliferation and apoptosis in vascular smooth muscle cells: relevance to the ADPKD phenotype. Circ Res 2005 1.01
67 Polycystin-1 maturation requires polycystin-2 in a dose-dependent manner. J Clin Invest 2015 1.00
68 Analysis of published PKD1 gene sequence variants. Nat Genet 2007 0.98
69 Transition fibre protein FBF1 is required for the ciliary entry of assembled intraflagellar transport complexes. Nat Commun 2013 0.97
70 Polycystin-2 mutations lead to impaired calcium cycling in the heart and predispose to dilated cardiomyopathy. J Mol Cell Cardiol 2013 0.95
71 A novel mutation of LAMB2 in a multigenerational mennonite family reveals a new phenotypic variant of Pierson syndrome. Ophthalmology 2011 0.94
72 Polycystic kidney disease in 2011: Connecting the dots toward a polycystic kidney disease therapy. Nat Rev Nephrol 2011 0.93
73 Epidermal growth factor receptor tyrosine kinase inhibition is not protective in PCK rats. Kidney Int 2004 0.92
74 PKHDL1, a homolog of the autosomal recessive polycystic kidney disease gene, encodes a receptor with inducible T lymphocyte expression. Hum Mol Genet 2003 0.92
75 Effect of calcium-sensing receptor activation in models of autosomal recessive or dominant polycystic kidney disease. Nephrol Dial Transplant 2008 0.92
76 Genetic variation of DKK3 may modify renal disease severity in ADPKD. J Am Soc Nephrol 2010 0.91
77 Disease stage characterization of hepatorenal fibrocystic pathology in the PCK rat model of ARPKD. Anat Rec (Hoboken) 2010 0.88
78 Functional characterization of PKDREJ, a male germ cell-restricted polycystin. J Cell Physiol 2006 0.87
79 Deficiency of polycystin-2 reduces Ca2+ channel activity and cell proliferation in ADPKD lymphoblastoid cells. FASEB J 2004 0.87
80 The mutation, a key determinant of phenotype in ADPKD. J Am Soc Nephrol 2013 0.87
81 Polycystin-1 regulates amphiregulin expression through CREB and AP1 signalling: implications in ADPKD cell proliferation. J Mol Med (Berl) 2012 0.85
82 The genetics of vascular complications in autosomal dominant polycystic kidney disease (ADPKD). Curr Hypertens Rev 2013 0.84
83 Insignificant effect of secretin in rodent models of polycystic kidney and liver disease. Am J Physiol Renal Physiol 2012 0.84
84 The Meckel syndrome protein meckelin (TMEM67) is a key regulator of cilia function but is not required for tissue planar polarity. Hum Mol Genet 2013 0.83
85 Endothelial dysfunction occurs prior to clinical evidence of polycystic kidney disease. Am J Nephrol 2013 0.82
86 Tuberous sclerosis complex and polycystic kidney disease together: an exception to the contiguous gene syndrome. Genet Med 2006 0.82
87 Germline PKHD1 mutations are protective against colorectal cancer. Hum Genet 2011 0.82
88 Investigation of primary cilia in the pathogenesis of biliary atresia. J Pediatr Gastroenterol Nutr 2011 0.82
89 Polycystic kidneys have decreased vascular density: a micro-CT study. Microcirculation 2013 0.81
90 NF-kappaB activation is required for apoptosis in fibrocystin/polyductin-depleted kidney epithelial cells. Apoptosis 2010 0.80
91 Molecular diagnostics of ADPKD coming of age. Clin J Am Soc Nephrol 2008 0.79
92 Mesenchymal Stromal Cells Improve Renovascular Function in Polycystic Kidney Disease. Cell Transplant 2014 0.77
93 Molecular analysis of a consanguineous Iranian polycystic kidney disease family identifies a PKD2 mutation that aids diagnostics. BMC Nephrol 2013 0.76
94 Somatotroph pituitary adenoma with acromegaly and autosomal dominant polycystic kidney disease: SSTR5 polymorphism and PKD1 mutation. Pituitary 2012 0.75
95 Differential expression of renal proteins in a rodent model of Meckel syndrome. Nephron Exp Nephrol 2010 0.75