PubRank

Gregory G Germino

Author PubWeight™ 72.58‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 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
2 A critical developmental switch defines the kinetics of kidney cyst formation after loss of Pkd1. Nat Med 2007 4.40
3 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
4 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
5 TRPP2 and TRPV4 form a polymodal sensory channel complex. J Cell Biol 2008 2.88
6 Cleavage of polycystin-1 requires the receptor for egg jelly domain and is disrupted by human autosomal-dominant polycystic kidney disease 1-associated mutations. Proc Natl Acad Sci U S A 2002 2.29
7 A functional floxed allele of Pkd1 that can be conditionally inactivated in vivo. J Am Soc Nephrol 2004 1.97
8 Molecular advances in autosomal dominant polycystic kidney disease. Adv Chronic Kidney Dis 2010 1.77
9 Rapamycin ameliorates PKD resulting from conditional inactivation of Pkd1. J Am Soc Nephrol 2010 1.70
10 Spectrum of mutations in the gene for autosomal recessive polycystic kidney disease (ARPKD/PKHD1). J Am Soc Nephrol 2003 1.70
11 T-cell factor/β-catenin activity is suppressed in two different models of autosomal dominant polycystic kidney disease. Kidney Int 2011 1.69
12 Polycystin 2 interacts with type I inositol 1,4,5-trisphosphate receptor to modulate intracellular Ca2+ signaling. J Biol Chem 2005 1.64
13 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
14 Intragenic motifs regulate the transcriptional complexity of Pkhd1/PKHD1. J Mol Med (Berl) 2014 1.56
15 Autosomal dominant polycystic kidney disease: molecular genetics and pathophysiology. J Lab Clin Med 2003 1.56
16 Essential role of cleavage of Polycystin-1 at G protein-coupled receptor proteolytic site for kidney tubular structure. Proc Natl Acad Sci U S A 2007 1.52
17 A Pkd1-Fbn1 genetic interaction implicates TGF-β signaling in the pathogenesis of vascular complications in autosomal dominant polycystic kidney disease. J Am Soc Nephrol 2013 1.49
18 Polycystin-1 induces cell migration by regulating phosphatidylinositol 3-kinase-dependent cytoskeletal rearrangements and GSK3beta-dependent cell cell mechanical adhesion. Mol Biol Cell 2007 1.47
19 Polyductin undergoes notch-like processing and regulated release from primary cilia. Hum Mol Genet 2007 1.44
20 Genetic interaction studies link autosomal dominant and recessive polycystic kidney disease in a common pathway. Hum Mol Genet 2007 1.43
21 Polyductin, the PKHD1 gene product, comprises isoforms expressed in plasma membrane, primary cilium, and cytoplasm. Kidney Int 2004 1.42
22 Identification and characterization of Pkhd1, the mouse orthologue of the human ARPKD gene. J Am Soc Nephrol 2002 1.41
23 PKHD1 mutations in autosomal recessive polycystic kidney disease (ARPKD). Hum Mutat 2004 1.40
24 Linking cilia to Wnts. Nat Genet 2005 1.40
25 Role of polycystins in renal tubulogenesis. Trends Cell Biol 2003 1.35
26 Loss of Bardet Biedl syndrome proteins causes defects in peripheral sensory innervation and function. Proc Natl Acad Sci U S A 2007 1.32
27 Evaluating the clinical utility of a molecular genetic test for polycystic kidney disease. Mol Genet Metab 2007 1.31
28 Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis: summary statement of a first National Institutes of Health/Office of Rare Diseases conference. J Pediatr 2006 1.26
29 Polycystin-1 induces resistance to apoptosis through the phosphatidylinositol 3-kinase/Akt signaling pathway. J Am Soc Nephrol 2006 1.23
30 Pkd1 haploinsufficiency increases renal damage and induces microcyst formation following ischemia/reperfusion. J Am Soc Nephrol 2009 1.19
31 Milder presentation of recessive polycystic kidney disease requires presence of amino acid substitution mutations. J Am Soc Nephrol 2003 1.13
32 mTOR inhibitors in polycystic kidney disease. N Engl J Med 2010 1.09
33 Expression of PKD1 and PKD2 transcripts and proteins in human embryo and during normal kidney development. Am J Pathol 2002 1.08
34 Polycystic kidney disease, cilia, and planar polarity. Methods Cell Biol 2009 1.00
35 Inactivation of Pkd1 in principal cells causes a more severe cystic kidney disease than in intercalated cells. Kidney Int 2009 0.95
36 Loss of polycystin-1 or polycystin-2 results in dysregulated apolipoprotein expression in murine tissues via alterations in nuclear hormone receptors. Hum Mol Genet 2005 0.94
37 Pkd1 and Pkd2 are required for normal placental development. PLoS One 2010 0.94
38 Network analysis of a Pkd1-mouse model of autosomal dominant polycystic kidney disease identifies HNF4α as a disease modifier. PLoS Genet 2012 0.90
39 The isolated polycystin-1 COOH-terminal can activate or block polycystin-1 signaling. Biochem Biophys Res Commun 2007 0.88
40 A regulatory role of polycystin-1 on cystic fibrosis transmembrane conductance regulator plasma membrane expression. Cell Physiol Biochem 2006 0.87
41 Heterologous expression of polycystin-1 inhibits endoplasmic reticulum calcium leak in stably transfected MDCK cells. Am J Physiol Renal Physiol 2008 0.84
42 Refinement of the autosomal recessive polycystic kidney disease (PKHD1) interval and exclusion of an EF hand-containing gene as a PKHD1 candidate gene. Am J Med Genet 2002 0.84
43 ARPKD and ADPKD: first cousins or more distant relatives? J Am Soc Nephrol 2008 0.81
44 Macromolecular assembly of polycystin-2 intracytosolic C-terminal domain. Proc Natl Acad Sci U S A 2011 0.81
45 Polycystin-1 regulates the stability and ubiquitination of transcription factor Jade-1. Hum Mol Genet 2012 0.81
46 A splice form of polycystin-2, lacking exon 7, does not interact with polycystin-1. Hum Mol Genet 2005 0.80
47 Ectopic expression of Cux1 is associated with reduced p27 expression and increased apoptosis during late stage cyst progression upon inactivation of Pkd1 in collecting ducts. Dev Dyn 2011 0.78
48 Progesterone induced mesenchymal differentiation and rescued cystic dilation of renal tubules of Pkd1(-/-) mice. Biochem Biophys Res Commun 2012 0.77
49 Polycystic kidney disease: Polycystin-1 and polycystin-2--it's complicated. Nat Rev Nephrol 2013 0.77
50 Systems biology of polycystic kidney disease: a critical review. Wiley Interdiscip Rev Syst Biol Med 2015 0.75