Research Topics
Genomes and Genes
| Patricia D WilsonSummaryAffiliation: Mount Sinai School of Medicine Country: USA Publications
Research Grants
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Detail Information
Publications
Polycystic kidney disease: new understanding in the pathogenesisPatricia D Wilson
Division of Nephrology, Department of Medicine, Mount Sinai School of Medicine, One Gustave L Levy Place, 1425 Madison Avenue, Box 1243, New York, NY 10029, USA
Int J Biochem Cell Biol 36:1868-73. 2004....- Cystic disease of the kidneyPatricia D Wilson
Division of Nephrology, Department of Medicine, Mount Sinai School of Medicine, New York, NY 10029, USA
Annu Rev Pathol 2:341-68. 2007.... - Inhibition of HER-2(neu/ErbB2) restores normal function and structure to polycystic kidney disease (PKD) epitheliaSamantha J Wilson
Department of Medicine, Mount Sinai School of Medicine, New York, NY 10029, USA
Biochim Biophys Acta 1762:647-55. 2006..These studies implicate HER2(neu/ErbB2) as an effector of apical EGFR complex mispolarization and that its inhibition should be considered a candidate for clinical therapy of ADPKD... - Polycystin: new aspects of structure, function, and regulationP D Wilson
Mount Sinai School of Medicine, 1425 Madison Avenue, New York, NY 10029, USA
J Am Soc Nephrol 12:834-45. 2001..Genetic evidence suggests that PKD1, PKD2, NPHP1, and tensin are in the same pathway... - Polycystic kidney diseasePatricia D Wilson
Department of Medicine, Division of Nephrology, Mount Sinai School of Medicine, New York, NY 10029, USA
N Engl J Med 350:151-64. 2004 - Branching morphogenesis of the ureteric epithelium during kidney development is coordinated by the opposing functions of GDNF and Sprouty1M Albert Basson
Division of Hematology Oncology, Mount Sinai School of Medicine, One Gustave L Levy Place, New York, NY 10029, USA
Dev Biol 299:466-77. 2006..Epithelial cysts develop in Spry1-deficient kidneys that share several molecular characteristics with those observed in human disease, suggesting that Spry1 null mice may be useful animal models for cystic hyperplasia... - Protein kinase X (PRKX) can rescue the effects of polycystic kidney disease-1 gene (PKD1) deficiencyXiaohong Li
Department of Medicine, Mount Sinai School of Medicine, New York, NY, USA
Biochim Biophys Acta 1782:1-9. 2008..These results suggest that PRKX can restore normal function to PKD1-deficient kidneys and have implications for the development of preventative therapy for ADPKD... 
Mechanoregulation of intracellular Ca2+ in human autosomal recessive polycystic kidney disease cyst-lining renal epithelial cellsRajeev Rohatgi
The Mount Sinai School of Medicine, One Gustave L Levy Place, Box 1243, New York, New York 10029, USA
Am J Physiol Renal Physiol 294:F890-9. 2008..In summary, human ARPKD cells exhibit an exaggerated Gd(3+)-sensitive mechano-induced Ca(2+) response compared with controls; whether this represents dysregulated polycystin-2 activity in ARPKD cells remains to be explored...
Abnormalities in focal adhesion complex formation, regulation, and function in human autosomal recessive polycystic kidney disease epithelial cellsSharon Israeli
Department of Medicine, Division of Nephrology, Mount Sinai School of Medicine, New York, New York, USA
Am J Physiol Cell Physiol 298:C831-46. 2010..Paxillin coimmunoprecipitation analyses suggested that fibrocystin-1 was a component of the normal focal adhesion complex and that actin and fibrocystin-1 were lost from ARPKD complexes...- Protein kinase-X interacts with Pin-1 and Polycystin-1 during mouse kidney developmentXiaohong Li
Department of Medicine, Mount Sinai School of Medicine, New York, New York 10029, USA
Kidney Int 76:54-62. 2009..These studies suggest that Polycystin-1 and Pin-1 may mediate the function of PRKX in kidney development... - Modification of the composition of polycystin-1 multiprotein complexes by calcium and tyrosine phosphorylationL Geng
Division of Nephrology, Department of Medicine, Mount Sinai School of Medicine, Box 1243, 1 Gustave L Levy Place, 10029, New York, NY 10029, USA
Biochim Biophys Acta 1535:21-35. 2000..Although inhibition of tyrosine phosphorylation by tyrphostin inhibits polycystin-1-FAK interactions, E-cadherin interactions are enhanced. High calcium treatment also increases polycystin-1-E-cadherin interactions... - Disruption of polycystin-1 function interferes with branching morphogenesis of the ureteric bud in developing mouse kidneysKatalin Polgar
Department of Medicine, Division of Nephrology, Mount Sinai School of Medicine, 1425 Madison Avenue, New York, NY 10029, USA
Dev Biol 286:16-30. 2005.... - Protein kinase X activates ureteric bud branching morphogenesis in developing mouse metanephric kidneyXiaohong Li
Department of Medicine, Division of Nephrology, Mount Sinai School of Medicine, New York, NY 10029, USA
J Am Soc Nephrol 16:3543-52. 2005..These results suggest that PRKX stimulates epithelial branching morphogenesis by activating cell migration and support a role for this kinase in the regulation of nephrogenesis and of collecting system development in the fetal kidney... - The exocyst localizes to the primary cilium in MDCK cellsKatherine K Rogers
Department of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
Biochem Biophys Res Commun 319:138-43. 2004..We further show that the exocyst is overexpressed in both cell lines and primary cell cultures of ADPKD origin, suggesting that the exocyst may be involved in the pathogenesis of ADPKD... - Polycystic kidney disease protein fibrocystin localizes to the mitotic spindle and regulates spindle bipolarityJingjing Zhang
Renal Division, Department of Medicine, Room 522, Harvard Institute of Medicine, 4 Blackfan Circle, Boston, MA 02115, USA
Hum Mol Genet 19:3306-19. 2010..These findings demonstrate a novel function of FPC in centrosome duplication and mitotic spindle assembly during cell division. We propose that mitotic defects due to FPC dysfunction contribute to cystogenesis in ARPKD... - PRKX critically regulates endothelial cell proliferation, migration, and vascular-like structure formationXiaohong Li
Department of Neurochemistry, NY State Institute for Basic Research in Developmental Disabilities, New York, NY 10314, USA
Dev Biol 356:475-85. 2011..The interaction of PRKX with Pin-1, Magi-1 and Bag-3 could contribute to the stimulating role of PRKX in angiogenesis... - PRKX, a phylogenetically and functionally distinct cAMP-dependent protein kinase, activates renal epithelial cell migration and morphogenesisXiaohong Li
Division of Nephrology, Department of Medicine, Mount Sinai School of Medicine, New York, NY 10029, USA
Proc Natl Acad Sci U S A 99:9260-5. 2002.... - The receptor tyrosine kinase regulator Sprouty1 is a target of the tumor suppressor WT1 and important for kidney developmentIsabelle Gross
Department of Medicine, Mount Sinai School of Medicine, New York, New York 10029, USA
J Biol Chem 278:41420-30. 2003..In addition, sprouty1 was expressed in the ureteric tree and antisense-treated ureteric trees had cystic lumens. Therefore, sprouty1 represents a physiologically relevant target gene of WT1 during kidney development... - Activator of G protein signaling 3 promotes epithelial cell proliferation in PKDRama Nadella
Department of Medicine, Medical College of Wisconsin, Milwaukee, WI 53226, USA
J Am Soc Nephrol 21:1275-80. 2010..AGS3 may play a receptor-independent role to regulate Galpha subunit function and control epithelial cell function in PKD... - Sprouty1 is a critical regulator of GDNF/RET-mediated kidney inductionM Albert Basson
Department of Medicine, Mount Sinai School of Medicine, One Gustave L Levy Place, New York, New York, USA
Dev Cell 8:229-39. 2005..These results demonstrate the importance of negative feedback regulation of RTK signaling during kidney induction and suggest that failures in feedback control may underlie some human congenital kidney malformations... - Mouse models of polycystic kidney diseasePatricia D Wilson
Mount Sinai School of Medicine, New York, USA
Curr Top Dev Biol 84:311-50. 2008..Appropriate genocopy models that faithfully recapitulate the phenotypic characteristics of the disease will be invaluable tools to analyze the effects of modifier genes and small molecule inhibitor therapies... - The autosomal recessive polycystic kidney disease protein is localized to primary cilia, with concentration in the basal body areaShixuan Wang
Renal Division, Department of Medicine, Brigham and Women s Hospital and Harvard Medical School, Boston, MA 02115, USA
J Am Soc Nephrol 15:592-602. 2004..It is proposed that the pathogenesis of autosomal recessive polycystic kidney disease is linked to the dysfunction of primary cilia... - Pathways of apoptosis in human autosomal recessive and autosomal dominant polycystic kidney diseasesBeatrice Goilav
Department of Pediatrics, Division of Pediatric Nephrology, Mount Sinai School of Medicine, New York, NY, USA
Pediatr Nephrol 23:1473-82. 2008..In conclusion, apoptosis in human ADPKD is an early event, occurring predominantly in normal-appearing tubules and small cysts, and is triggered by an extrinsic factor, but it occurs later in ARPKD... - c-Src inactivation reduces renal epithelial cell-matrix adhesion, proliferation, and cyst formationJustine Elliott
Division of Nephrology, Mount Sinai School of Medicine, New York, New York, USA
Am J Physiol Cell Physiol 301:C522-9. 2011..This suggests that SKI-606 has dual effects on cystic epithelial cell proliferation and ECM adhesion and may have therapeutic potential for ADPKD patients... - Formation of 20-hydroxyeicosatetraenoic acid, a vasoactive and natriuretic eicosanoid, in human kidney. Role of Cyp4F2 and Cyp4A11J M Lasker
Department of Biochemistry, Mount Sinai School of Medicine, New York, New York 10029, USA
J Biol Chem 275:4118-26. 2000..Considering their proximal tubular localization, these P450 enzymes may partake in pivotal renal functions, including the regulation of salt and water balance, and arterial blood pressure itself... - Apical plasma membrane mispolarization of NaK-ATPase in polycystic kidney disease epithelia is associated with aberrant expression of the beta2 isoformP D Wilson
Department of Medicine, Mount Sinai School of Medicine, New York, New York 10029, USA
Am J Pathol 156:253-68. 2000..These results are consistent with a model in which the additional transcription and translation of the beta2 subunit of NaK-ATPase may result in the apical mislocalization of NaK-ATPase in ADPKD cystic epithelia... - Microcyst formation and HIV-1 gene expression occur in multiple nephron segments in HIV-associated nephropathyM J Ross
Division of Nephrology, Mount Sinai School of Medicine, New York, New York, USA
J Am Soc Nephrol 12:2645-51. 2001..These data support a direct role for HIV-1 infection of renal epithelial cells in the pathogenesis of microcyst formation in patients with HIVAN... - Expression of the beta2-subunit and apical localization of Na+-K+-ATPase in metanephric kidneyC R Burrow
Division of Nephrology, Department of Medicine, Mount Sinai School of Medicine, New York, New York 10029, USA
Am J Physiol 277:F391-403. 1999.... - Cystic fibrosis transmembrane conductance regulator in the kidney: clues to its role?P D Wilson
Mount Sinai School of Medicine, New York, NY 10029, USA
Exp Nephrol 7:284-9. 1999..Similar colocalization of WT-CFTR and Na-K-ATPase at the apical plasma membranes is found in collecting tubules during development when it is speculated to play a role in the initiation of opening of the tubule lumen... - Identification of phosphorylation sites in the PKD1-encoded protein C-terminal domainH P Li
Department of Medicine, Mount Sinai School of Medicine, New York, New York 10029, USA
Biochem Biophys Res Commun 259:356-63. 1999..In addition, we suggest that Y4237 might be phosphorylated by c-src in living cells... - Cystic diseases of the kidney: role of adhesion molecules in normal and abnormal tubulogenesisP D Wilson
Mount Sinai School of Medicine, New York, N Y, USA
Exp Nephrol 7:114-24. 1999..Further evidence of a critical role of cell-matrix interactions via focal adhesion complex formation is provided by the development of renal cystic disease in tensin knockout mice... - The PKD1 gene product, "polycystin-1," is a tyrosine-phosphorylated protein that colocalizes with alpha2beta1-integrin in focal clusters in adherent renal epitheliaP D Wilson
Department of Medicine, Mount Sinai School of Medicine, New York, New York 10029, USA
Lab Invest 79:1311-23. 1999..These studies suggest that the PKD1-encoded protein is part of a large multiprotein complex in epithelial cells that functions in the regulation of extracellular matrix interactions with the plasma membrane and cell cytoskeleton... - Stable knockdown of polycystin-1 confers integrin-alpha2beta1-mediated anoikis resistanceLorenzo Battini
Division of Renal Medicine, Mount Sinai School of Medicine, New York, NY 10029, USA
J Am Soc Nephrol 17:3049-58. 2006..Furthermore, these findings indicate that reduction of PC1 expression levels, rather than the loss of heterozygosity, may be sufficient to induce cystogenesis... - Role of ubiquitin-like protein FAT10 in epithelial apoptosis in renal diseaseMichael J Ross
Mount Sinai School of Medicine, Division of Nephrology, Box 1243, One Gustave L Levy Place, New York, NY 10029, USA
J Am Soc Nephrol 17:996-1004. 2006..These results suggest a novel role for FAT10 in epithelial apoptosis, which is an important component of the pathogenesis of many renal diseases... - Expression of the urate transporter/channel is developmentally regulated in human kidneysD P Hyink
Division of Nephrology, Department of Medicine, Mount Sinai School of Medicine, New York, New York 10029, USA
Am J Physiol Renal Physiol 281:F875-86. 2001..These data imply that different developmental factors regulate the initiation of cytoplasmic hUAT expression and subsequent insertion into human proximal tubule brush-border membranes... - Stem cells and kidney injuryCecile Vigneau
Mount Sinai School of Medicine, Department of Medicine, New York, New York 10029, USA
Curr Opin Nephrol Hypertens 15:238-44. 2006..Adult stem cells in the bone marrow or in a specific kidney niche may provide a source of stem cells with a therapeutic potential... - Na transport in autosomal recessive polycystic kidney disease (ARPKD) cyst lining epithelial cellsRajeev Rohatgi
Division of Renal Medicine, Mount Sinai School of Medicine, New York, New York 10029, USA
J Am Soc Nephrol 14:827-36. 2003..Whether Na absorption is mediated by ENaC, perhaps of nonclassical subunit composition, or another amiloride-sensitive transporter remains to be determined... - The genes and proteins associated with poly-cystic kidney diseasesP D Wilson
Department of Medicine, Mount Sinai School of Medicine, Box 1243, 1425 Madison Avenue, New York, NY 10029, USA
Minerva Urol Nefrol 54:201-11. 2002..It is suggested that the function of the polycystin complex is to act as a key developmental regulator of renal tubule morphogenesis... - WT1 expression induces features of renal epithelial differentiation in mesenchymal fibroblastsS Hosono
Brookdale Center for Developmental and Molecular Biology and Department of Medicine, Mount Sinai School of Medicine, New York, NY 10029, USA
Oncogene 18:417-27. 1999.... - Polarity of alpha-galactosidase A uptake by renal tubule cellsAaron S Stern
Department of Medicine, Mount Sinai School of Medicine, New York, New York 10029, USA
Kidney Int 61:S52-5. 2002..This will likely best be achieved with local administration of a vector containing the transgene directly to the kidney... - Loss of chromosome 16 from renal epithelial cells in humansJeffrey P Gardner
Hypertension Research Center, University of Medicine and Dentistry of New Jersey, New Jersey Medical School, Newark, NJ 07103-2714, USA
Hypertension 40:928-33. 2002.... - Comparative ontogeny, processing, and segmental distribution of the renal chloride channel, ClC-5Francois Jouret
Division of Nephrology and Laboratory of Developmental Genetics, Universite Catholique de Louvain, Brussels, Belgium
Kidney Int 65:198-208. 2004..However, the ontogeny of ClC-5, and its processing and segmental distribution with respect to related proteins during nephrogenesis remain unknown...
Research Grants
- Novel Therapeutic Strategies for PKDPatricia Wilson; Fiscal Year: 2007..abstract_text> ..