Published in J Am Soc Nephrol on May 22, 2014
Polycystic Kidney Disease with Hyperinsulinemic Hypoglycemia Caused by a Promoter Mutation in Phosphomannomutase 2. J Am Soc Nephrol (2017) 1.44
A polycystin-centric view of cyst formation and disease: the polycystins revisited. Kidney Int (2015) 1.01
Aquaporin 11, a regulator of water efflux at retinal Müller glial cell surface decreases concomitant with immune-mediated gliosis. J Neuroinflammation (2016) 0.80
Genetic predisposition of donors affects the allograft outcome in kidney transplantation: Single-nucleotide polymorphism of aquaporin-11. Kidney Res Clin Pract (2015) 0.78
Abnormal glycosylation in Joubert syndrome type 10. Cilia (2017) 0.77
Aquaporin-11 (AQP11) Expression in the Mouse Brain. Int J Mol Sci (2016) 0.76
Enhanced Autophagy in Polycystic Kidneys of AQP11 Null Mice. Int J Mol Sci (2016) 0.75
Proteomic analysis of AQP11-null kidney: Proximal tubular type polycystic kidney disease. Biochem Biophys Rep (2017) 0.75
Simple and highly efficient BAC recombineering using galK selection. Nucleic Acids Res (2005) 11.23
Chlamydomonas IFT88 and its mouse homologue, polycystic kidney disease gene tg737, are required for assembly of cilia and flagella. J Cell Biol (2000) 7.04
The polycystic kidney disease proteins, polycystin-1, polycystin-2, polaris, and cystin, are co-localized in renal cilia. J Am Soc Nephrol (2002) 5.58
Kidney-specific inactivation of the KIF3A subunit of kinesin-II inhibits renal ciliogenesis and produces polycystic kidney disease. Proc Natl Acad Sci U S A (2003) 4.47
Somatic inactivation of Pkd2 results in polycystic kidney disease. Cell (1998) 3.84
A genetic interaction network of five genes for human polycystic kidney and liver diseases defines polycystin-1 as the central determinant of cyst formation. Nat Genet (2011) 3.18
Polycystin-1, STAT6, and P100 function in a pathway that transduces ciliary mechanosensation and is activated in polycystic kidney disease. Dev Cell (2006) 2.64
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
Cardiac defects and renal failure in mice with targeted mutations in Pkd2. Nat Genet (2000) 2.24
Mechanical stimuli induce cleavage and nuclear translocation of the polycystin-1 C terminus. J Clin Invest (2004) 2.00
Polycystins and primary cilia: primers for cell cycle progression. Annu Rev Physiol (2009) 1.99
Defects in cholangiocyte fibrocystin expression and ciliary structure in the PCK rat. Gastroenterology (2003) 1.80
Development of polycystic kidney disease in juvenile cystic kidney mice: insights into pathogenesis, ciliary abnormalities, and common features with human disease. J Am Soc Nephrol (2006) 1.78
Polycystin: in vitro synthesis, in vivo tissue expression, and subcellular localization identifies a large membrane-associated protein. Proc Natl Acad Sci U S A (1997) 1.75
Functional polycystin-1 dosage governs autosomal dominant polycystic kidney disease severity. J Clin Invest (2012) 1.74
Nek8 regulates the expression and localization of polycystin-1 and polycystin-2. J Am Soc Nephrol (2008) 1.70
Identification, characterization, and localization of a novel kidney polycystin-1-polycystin-2 complex. J Biol Chem (2002) 1.56
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
Somatic PKD2 mutations in individual kidney and liver cysts support a "two-hit" model of cystogenesis in type 2 autosomal dominant polycystic kidney disease. J Am Soc Nephrol (1999) 1.50
Disruption of aquaporin-11 produces polycystic kidneys following vacuolization of the proximal tubule. Mol Cell Biol (2005) 1.48
Impaired urea accumulation in the inner medulla of mice lacking the urea transporter UT-A2. Mol Cell Biol (2005) 1.47
Pioglitazone improves the phenotype and molecular defects of a targeted Pkd1 mutant. Hum Mol Genet (2002) 1.43
A polycystin-1 multiprotein complex is disrupted in polycystic kidney disease cells. Mol Biol Cell (2004) 1.39
Polycystin-1 distribution is modulated by polycystin-2 expression in mammalian cells. J Biol Chem (2003) 1.38
In vivo protein biotinylation for identification of organ-specific antigens accessible from the vasculature. Nat Methods (2005) 1.32
Aquaporin-11 containing a divergent NPA motif has normal water channel activity. Biochim Biophys Acta (2006) 1.15
Acute insulin stimulation induces phosphorylation of the Na-Cl cotransporter in cultured distal mpkDCT cells and mouse kidney. PLoS One (2011) 1.13
Single amino acid substitution in aquaporin 11 causes renal failure. J Am Soc Nephrol (2008) 1.09
Pathogenesis and treatment of autosomal-dominant nephrogenic diabetes insipidus caused by an aquaporin 2 mutation. Proc Natl Acad Sci U S A (2006) 1.09
Congenital disorders of glycosylation in hepatology: the example of polycystic liver disease. J Hepatol (2009) 1.06
Aquaporin-11 knockout mice and polycystic kidney disease animals share a common mechanism of cyst formation. FASEB J (2008) 1.04
Pkd1 transgenic mice: adult model of polycystic kidney disease with extrarenal and renal phenotypes. Hum Mol Genet (2010) 1.04
Water permeability and characterization of aquaporin-11. J Struct Biol (2011) 1.04
The NPC motif of aquaporin-11, unlike the NPA motif of known aquaporins, is essential for full expression of molecular function. J Biol Chem (2010) 1.01
A loss-of-function model for cystogenesis in human autosomal dominant polycystic kidney disease type 2. Am J Hum Genet (1999) 0.99
Phosphatidylinositol 3-kinase/Akt signaling pathway activates the WNK-OSR1/SPAK-NCC phosphorylation cascade in hyperinsulinemic db/db mice. Hypertension (2012) 0.99
PKD1 haploinsufficiency causes a syndrome of inappropriate antidiuresis in mice. J Am Soc Nephrol (2007) 0.97
Liver-specific Aquaporin 11 knockout mice show rapid vacuolization of the rough endoplasmic reticulum in periportal hepatocytes after amino acid feeding. Am J Physiol Gastrointest Liver Physiol (2012) 0.96
Polycystin-1 surface localization is stimulated by polycystin-2 and cleavage at the G protein-coupled receptor proteolytic site. Mol Biol Cell (2010) 0.96
Polycystic kidneys caused by sustained expression of Cux1 isoform p75. J Biol Chem (2008) 0.91
Aquaporin 11 insufficiency modulates kidney susceptibility to oxidative stress. Am J Physiol Renal Physiol (2013) 0.87
Pancreas-specific aquaporin 12 null mice showed increased susceptibility to caerulein-induced acute pancreatitis. Am J Physiol Cell Physiol (2009) 0.85