Published in J Am Soc Nephrol on February 16, 2012
Diagnosis and management of polycystic liver disease. Nat Rev Gastroenterol Hepatol (2013) 1.46
Looking at the (w)hole: magnet resonance imaging in polycystic kidney disease. Pediatr Nephrol (2012) 0.97
Rheb/mTORC1 signaling promotes kidney fibroblast activation and fibrosis. J Am Soc Nephrol (2013) 0.95
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
The Future of Polycystic Kidney Disease Research--As Seen By the 12 Kaplan Awardees. J Am Soc Nephrol (2015) 0.93
Skin cancer in solid organ transplant recipients: are mTOR inhibitors a game changer? Transplant Res (2015) 0.91
Low-dose rapamycin (sirolimus) effects in autosomal dominant polycystic kidney disease: an open-label randomized controlled pilot study. Clin J Am Soc Nephrol (2014) 0.90
New treatments for autosomal dominant polycystic kidney disease. Br J Clin Pharmacol (2013) 0.85
The role of phospholipase D in modulating the MTOR signaling pathway in polycystic kidney disease. PLoS One (2013) 0.82
Roles of mTOR complexes in the kidney: implications for renal disease and transplantation. Nat Rev Nephrol (2016) 0.82
Scattered Deletion of PKD1 in Kidneys Causes a Cystic Snowball Effect and Recapitulates Polycystic Kidney Disease. J Am Soc Nephrol (2014) 0.80
Targeting of receptor for advanced glycation end products suppresses cyst growth in polycystic kidney disease. J Biol Chem (2014) 0.77
Experimental therapies and ongoing clinical trials to slow down progression of ADPKD. Curr Hypertens Rev (2013) 0.76
Novel therapeutic approaches to autosomal dominant polycystic kidney disease. Transl Res (2014) 0.76
Variable Cyst Development in Autosomal Dominant Polycystic Kidney Disease: The Biologic Context. J Am Soc Nephrol (2016) 0.76
Mammalian target of rapamycin inhibition in polycystic kidney disease: From bench to bedside. Kidney Res Clin Pract (2012) 0.75
Gα12 is required for renal cystogenesis induced by Pkd1 inactivation. J Cell Sci (2016) 0.75
Polycystin-1 but not polycystin-2 deficiency causes upregulation of the mTOR pathway and can be synergistically targeted with rapamycin and metformin. Pflugers Arch (2013) 0.75
Autopsy report with clinical and pathophysiologic discussion of autosomal dominant adult polycystic kidney disease. Case Rep Urol (2014) 0.75
Absence of mTOR Inhibitor Effect on Hepatic Cyst Growth: A Case Report of a Kidney Transplant Recipient with Autosomal Dominant Polycystic Kidney Disease. Case Rep Transplant (2012) 0.75
Effects of TORC1 Inhibition during the Early and Established Phases of Polycystic Kidney Disease. PLoS One (2016) 0.75
Rapamycin reversal of VEGF-C-driven lymphatic anomalies in the respiratory tract. JCI Insight (2017) 0.75
Effects of PEP-1-FK506BP on cyst formation in polycystic kidney disease. BMB Rep (2017) 0.75
High-Throughput Phenotypic Screening of Kinase Inhibitors to Identify Drug Targets for Polycystic Kidney Disease. SLAS Discov (2017) 0.75
Rapamycin treatment dose-dependently improves the cystic kidney in a new ADPKD mouse model via the mTORC1 and cell-cycle-associated CDK1/cyclin axis. J Cell Mol Med (2017) 0.75
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Polycystins 1 and 2 mediate mechanosensation in the primary cilium of kidney cells. Nat Genet (2003) 13.33
Polycystic kidney disease. N Engl J Med (2004) 5.73
Everolimus in patients with autosomal dominant polycystic kidney disease. N Engl J Med (2010) 5.50
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
Sirolimus and kidney growth in autosomal dominant polycystic kidney disease. N Engl J Med (2010) 4.36
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
Polycystin-1 and -2 dosage regulates pressure sensing. Cell (2009) 3.36
mTOR and cancer: many loops in one pathway. Curr Opin Cell Biol (2009) 3.22
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
Rapamycin markedly slows disease progression in a rat model of polycystic kidney disease. J Am Soc Nephrol (2004) 2.69
Volume progression in autosomal dominant polycystic kidney disease: the major factor determining clinical outcomes. Clin J Am Soc Nephrol (2005) 2.66
Lowering of Pkd1 expression is sufficient to cause polycystic kidney disease. Hum Mol Genet (2004) 2.47
Sirolimus therapy to halt the progression of ADPKD. J Am Soc Nephrol (2010) 2.42
Lanreotide reduces the volume of polycystic liver: a randomized, double-blind, placebo-controlled trial. Gastroenterology (2009) 2.33
Cyclic AMP activates B-Raf and ERK in cyst epithelial cells from autosomal-dominant polycystic kidneys. Kidney Int (2003) 2.32
Sirolimus reduces polycystic liver volume in ADPKD patients. J Am Soc Nephrol (2008) 2.26
Kidney-specific inactivation of the Pkd1 gene induces rapid cyst formation in developing kidneys and a slow onset of disease in adult mice. Hum Mol Genet (2007) 2.03
Rapamycin ameliorates PKD resulting from conditional inactivation of Pkd1. J Am Soc Nephrol (2010) 1.70
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
The polycystic kidney disease 1 gene product mediates protein kinase C alpha-dependent and c-Jun N-terminal kinase-dependent activation of the transcription factor AP-1. J Biol Chem (1998) 1.57
Incompletely penetrant PKD1 alleles mimic the renal manifestations of ARPKD. J Am Soc Nephrol (2010) 1.52
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
Polycystin-1 induces resistance to apoptosis through the phosphatidylinositol 3-kinase/Akt signaling pathway. J Am Soc Nephrol (2006) 1.23
Polycystin-1, the product of the polycystic kidney disease 1 gene, co-localizes with desmosomes in MDCK cells. Hum Mol Genet (2000) 1.20
Curcumin inhibits cystogenesis by simultaneous interference of multiple signaling pathways: in vivo evidence from a Pkd1-deletion model. Am J Physiol Renal Physiol (2011) 1.17
Long-term rapamycin therapy in the Han:SPRD rat model of polycystic kidney disease (PKD). Nephrol Dial Transplant (2009) 1.16
Transgenic mice expressing tamoxifen-inducible Cre for somatic gene modification in renal epithelial cells. Genesis (2006) 1.14
Elevated TGFbeta-Smad signalling in experimental Pkd1 models and human patients with polycystic kidney disease. J Pathol (2010) 1.12
Rapamycin inhibits hepatic fibrosis in rats by attenuating multiple profibrogenic pathways. Liver Transpl (2009) 1.05
Therapeutic mTOR inhibition in autosomal dominant polycystic kidney disease: What is the appropriate serum level? Am J Transplant (2010) 1.03
Sirolimus attenuates disease progression in an orthologous mouse model of human autosomal dominant polycystic kidney disease. Kidney Int (2010) 1.02
Pulse mTOR inhibitor treatment effectively controls cyst growth but leads to severe parenchymal and glomerular hypertrophy in rat polycystic kidney disease. Am J Physiol Renal Physiol (2009) 0.98
Tight junction composition is altered in the epithelium of polycystic kidneys. J Pathol (2008) 0.95
Early and late effects of the immunosuppressants rapamycin and mycophenolate mofetil on UV carcinogenesis. Int J Cancer (2010) 0.89
Steroid and oxygen effects on eIF4F complex, mTOR, and ENaC translation in fetal lung epithelia. Am J Respir Cell Mol Biol (2007) 0.87
Mammalian target of rapamycin and autosomal dominant polycystic kidney disease. Transplant Proc (2009) 0.82
Therapeutic role of sirolimus in non-transplant kidney disease. Pharmacol Ther (2009) 0.78
Rapamycin inhibits primary and metastatic tumor growth by antiangiogenesis: involvement of vascular endothelial growth factor. Nat Med (2002) 7.06
Pathologic classification of diabetic nephropathy. J Am Soc Nephrol (2010) 3.61
Polycystin-1 and -2 dosage regulates pressure sensing. Cell (2009) 3.36
Cancer risk in hereditary nonpolyposis colorectal cancer due to MSH6 mutations: impact on counseling and surveillance. Gastroenterology (2004) 2.90
Role of truncating mutations in MME gene in fetomaternal alloimmunisation and antenatal glomerulopathies. Lancet (2004) 2.82
Compound inheritance of a low-frequency regulatory SNP and a rare null mutation in exon-junction complex subunit RBM8A causes TAR syndrome. Nat Genet (2012) 2.80
Mutations in SWI/SNF chromatin remodeling complex gene ARID1B cause Coffin-Siris syndrome. Nat Genet (2012) 2.63
Mutations in genes encoding subunits of RNA polymerases I and III cause Treacher Collins syndrome. Nat Genet (2010) 2.52
Lowering of Pkd1 expression is sufficient to cause polycystic kidney disease. Hum Mol Genet (2004) 2.47
Genetic heterogeneity in Rubinstein-Taybi syndrome: mutations in both the CBP and EP300 genes cause disease. Am J Hum Genet (2005) 2.40
Dynamic imaging of cancer growth and invasion: a modified skin-fold chamber model. Histochem Cell Biol (2008) 2.39
Peters Plus syndrome is caused by mutations in B3GALTL, a putative glycosyltransferase. Am J Hum Genet (2006) 2.31
Rubinstein-Taybi syndrome: clinical and molecular overview. Expert Rev Mol Med (2007) 2.17
Deep sequencing to reveal new variants in pooled DNA samples. Hum Mutat (2009) 2.04
Kidney-specific inactivation of the Pkd1 gene induces rapid cyst formation in developing kidneys and a slow onset of disease in adult mice. Hum Mol Genet (2007) 2.03
Two-color multiplex ligation-dependent probe amplification: detecting genomic rearrangements in hereditary multiple exostoses. Hum Mutat (2004) 1.94
Copy number variation in regions flanked (or unflanked) by duplicons among patients with developmental delay and/or congenital malformations; detection of reciprocal and partial Williams-Beuren duplications. Eur J Hum Genet (2006) 1.93
Interleukin 21 controls tumour growth and tumour immunosurveillance in colitis-associated tumorigenesis in mice. Gut (2011) 1.84
Carnosine as a protective factor in diabetic nephropathy: association with a leucine repeat of the carnosinase gene CNDP1. Diabetes (2005) 1.75
Identification of a human CD8+ regulatory T cell subset that mediates suppression through the chemokine CC chemokine ligand 4. Proc Natl Acad Sci U S A (2007) 1.75
Chimerism in kidneys, livers and hearts of normal women: implications for transplantation studies. Am J Transplant (2005) 1.74
Pericentrin forms a complex with intraflagellar transport proteins and polycystin-2 and is required for primary cilia assembly. J Cell Biol (2004) 1.73
Induction of IL-13 triggers TGF-beta1-dependent tissue fibrosis in chronic 2,4,6-trinitrobenzene sulfonic acid colitis. J Immunol (2007) 1.69
Pro- and anti-cancer effects of immunosuppressive agents used in organ transplantation. Transplantation (2004) 1.68
Copeptin, a surrogate marker for vasopressin, is associated with kidney function decline in subjects with autosomal dominant polycystic kidney disease. Nephrol Dial Transplant (2012) 1.67
Molecular diagnostics of Meckel-Gruber syndrome highlights phenotypic differences between MKS1 and MKS3. Hum Genet (2007) 1.64
Immunomodulatory effects of mesenchymal stem cells in a rat organ transplant model. Transplantation (2006) 1.64
Comprehensive detection of genomic duplications and deletions in the DMD gene, by use of multiplex amplifiable probe hybridization. Am J Hum Genet (2002) 1.55
Gene expression profiling in glomeruli from human kidneys with diabetic nephropathy. Am J Kidney Dis (2004) 1.53
Toxic tubular injury in kidneys from Pkd1-deletion mice accelerates cystogenesis accompanied by dysregulated planar cell polarity and canonical Wnt signaling pathways. Hum Mol Genet (2009) 1.53
The pathology of fresh and cryopreserved homograft heart valves: an analysis of forty explanted homograft valves. J Thorac Cardiovasc Surg (2002) 1.52
IL-13 signaling via IL-13R alpha2 induces major downstream fibrogenic factors mediating fibrosis in chronic TNBS colitis. Gastroenterology (2008) 1.51
Macrophage migration inhibitory factor promotes cyst growth in polycystic kidney disease. J Clin Invest (2015) 1.51
High volume naked DNA tail-vein injection restores liver function in Fah-knock out mice. J Gastroenterol Hepatol (2010) 1.42
Rapamycin protects allografts from rejection while simultaneously attacking tumors in immunosuppressed mice. Transplantation (2004) 1.40
Integrin signaling via RGD peptides and anti-beta1 antibodies confers resistance to apoptosis in islets of Langerhans. Diabetes (2006) 1.38
Gene expression profile and histopathology of experimental bronchopulmonary dysplasia induced by prolonged oxidative stress. Free Radic Biol Med (2004) 1.38
Heterozygous mutations in PMS2 cause hereditary nonpolyposis colorectal carcinoma (Lynch syndrome). Gastroenterology (2006) 1.34
Loss-of-function mutations in IGSF1 cause an X-linked syndrome of central hypothyroidism and testicular enlargement. Nat Genet (2012) 1.34
Increased risk of cancer other than melanoma in CDKN2A founder mutation (p16-Leiden)-positive melanoma families. Clin Cancer Res (2008) 1.32
Mammalian target of rapamycin is activated in human gastric cancer and serves as a target for therapy in an experimental model. Int J Cancer (2007) 1.30
Inhibition of heat shock protein 90 impairs epidermal growth factor-mediated signaling in gastric cancer cells and reduces tumor growth and vascularization in vivo. Mol Cancer Ther (2007) 1.29
Diagnostic approach and management of Lynch syndrome (hereditary nonpolyposis colorectal carcinoma): a guide for clinicians. CA Cancer J Clin (2006) 1.29
Inhibition of SRC tyrosine kinase as treatment for human pancreatic cancer growing orthotopically in nude mice. Clin Cancer Res (2004) 1.28
Standardization of whole blood immune phenotype monitoring for clinical trials: panels and methods from the ONE study. Transplant Res (2013) 1.27
Coffin-Siris syndrome and the BAF complex: genotype-phenotype study in 63 patients. Hum Mutat (2013) 1.27
Targeting FGFR/PDGFR/VEGFR impairs tumor growth, angiogenesis, and metastasis by effects on tumor cells, endothelial cells, and pericytes in pancreatic cancer. Mol Cancer Ther (2011) 1.26
Pathogenic sequence for dissecting aneurysm formation in a hypomorphic polycystic kidney disease 1 mouse model. Arterioscler Thromb Vasc Biol (2007) 1.26
Restoration of tumor immunosurveillance via targeting of interleukin-13 receptor-alpha 2. Cancer Res (2008) 1.26
Cutting Edge: Immunological consequences and trafficking of human regulatory macrophages administered to renal transplant recipients. J Immunol (2011) 1.25
Tumor development in murine ulcerative colitis depends on MyD88 signaling of colonic F4/80+CD11b(high)Gr1(low) macrophages. J Clin Invest (2011) 1.25
A cell-based approach to the minimization of immunosuppression in renal transplantation. Transpl Int (2008) 1.24
Genetic heterogeneity in Rubinstein-Taybi syndrome: delineation of the phenotype of the first patients carrying mutations in EP300. J Med Genet (2007) 1.23
Genetic testing in familial melanoma: uptake and implications. Psychooncology (2008) 1.21
GFP-transfected tumor cells are useful in examining early metastasis in vivo, but immune reaction precludes long-term tumor development studies in immunocompetent mice. Clin Exp Metastasis (2003) 1.19
Rapamycin induces tumor-specific thrombosis via tissue factor in the presence of VEGF. Blood (2005) 1.17
Transplant acceptance-inducing cells as an immune-conditioning therapy in renal transplantation. Transpl Int (2008) 1.17
Curcumin inhibits cystogenesis by simultaneous interference of multiple signaling pathways: in vivo evidence from a Pkd1-deletion model. Am J Physiol Renal Physiol (2011) 1.17
Toward MSC in solid organ transplantation: 2008 position paper of the MISOT study group. Transplantation (2009) 1.16
Loss of CBP acetyltransferase activity by PHD finger mutations in Rubinstein-Taybi syndrome. Hum Mol Genet (2003) 1.16
Association of urinary biomarkers with disease severity in patients with autosomal dominant polycystic kidney disease: a cross-sectional analysis. Am J Kidney Dis (2010) 1.15
Evaluation of diagnostic NOTCH3 immunostaining in CADASIL. Acta Neuropathol (2003) 1.14
IFN-γ-induced iNOS expression in mouse regulatory macrophages prolongs allograft survival in fully immunocompetent recipients. Mol Ther (2012) 1.14
Transgenic mice expressing tamoxifen-inducible Cre for somatic gene modification in renal epithelial cells. Genesis (2006) 1.14
Deletion of Foxp3+ regulatory T cells in genetically targeted mice supports development of intestinal inflammation. BMC Gastroenterol (2012) 1.13
Activating transcription factor-3 (ATF3) functions as a tumor suppressor in colon cancer and is up-regulated upon heat-shock protein 90 (Hsp90) inhibition. BMC Cancer (2010) 1.12
Craniosynostosis and multiple skeletal anomalies in humans and zebrafish result from a defect in the localized degradation of retinoic acid. Am J Hum Genet (2011) 1.12
Detecting copy number changes in genomic DNA: MAPH and MLPA. Methods Cell Biol (2004) 1.12
Distinct subcellular expression of endogenous polycystin-2 in the plasma membrane and Golgi apparatus of MDCK cells. Hum Mol Genet (2002) 1.12
Elevated TGFbeta-Smad signalling in experimental Pkd1 models and human patients with polycystic kidney disease. J Pathol (2010) 1.12
Dosing of rapamycin is critical to achieve an optimal antiangiogenic effect against cancer. Transpl Int (2005) 1.11
Potential for glomerular C4d as an indicator of thrombotic microangiopathy in lupus nephritis. Arthritis Rheum (2008) 1.11
Fidelity and evolution of recurrent FSGS in renal allografts. J Am Soc Nephrol (2008) 1.11
Unclassified variants in disease-causing genes: nonuniformity of genetic testing and counselling, a proposal for guidelines. Eur J Hum Genet (2005) 1.10