polycystic kidney diseases

Summary

Summary: Hereditary diseases that are characterized by the progressive expansion of a large number of tightly packed CYSTS within the KIDNEYS. They include diseases with autosomal dominant and autosomal recessive inheritance.

Top Publications

  1. ncbi Intraflagellar transport
    Joel L Rosenbaum
    Department of Molecular, Cellular and Developmental Biology, Yale University, New Haven, Connecticut 06520, USA
    Nat Rev Mol Cell Biol 3:813-25. 2002
  2. pmc MKS and NPHP modules cooperate to establish basal body/transition zone membrane associations and ciliary gate function during ciliogenesis
    Corey L Williams
    Department of Cell Biology, University of Alabama, Birmingham, AL 35294, USA
    J Cell Biol 192:1023-41. 2011
  3. pmc Polycystic kidney disease
    Peter C Harris
    Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota 55905, USA
    Annu Rev Med 60:321-37. 2009
  4. ncbi The primary cilium as the cell's antenna: signaling at a sensory organelle
    Veena Singla
    Program in Developmental and Stem Cell Biology, and Diabetes Center, University of California, San Francisco, CA 94143 0525, USA
    Science 313:629-33. 2006
  5. pmc Cyclic AMP-mediated cyst expansion
    Darren P Wallace
    Department of Internal Medicine, University of Kansas Medical Center, Kansas City, KS, USA
    Biochim Biophys Acta 1812:1291-300. 2011
  6. ncbi Polycystic kidney disease
    Patricia 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
  7. pmc Systems biology approach to identify transcriptome reprogramming and candidate microRNA targets during the progression of polycystic kidney disease
    Priyanka Pandey
    Department of Medicine, Children s Hospital Boston Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA
    BMC Syst Biol 5:56. 2011
  8. ncbi Epithelial-to-mesenchymal transition in cyst lining epithelial cells in an orthologous PCK rat model of autosomal-recessive polycystic kidney disease
    Hiroko Togawa
    Department of Pediatrics, Wakayama Medical University, Wakayama, Japan
    Am J Physiol Renal Physiol 300:F511-20. 2011
  9. pmc The mTOR pathway is regulated by polycystin-1, and its inhibition reverses renal cystogenesis in polycystic kidney disease
    Jonathan M Shillingford
    Department of Molecular, Cellular, and Developmental Biology, University of California, Santa Barbara, CA 93106 9610, USA
    Proc Natl Acad Sci U S A 103:5466-71. 2006
  10. ncbi Polycystic kidney disease and therapeutic approaches
    Eun Young Park
    Department of Biological Science, Sookmyung Women s University, Seoul 140 742, Korea
    BMB Rep 44:359-68. 2011

Detail Information

Publications221 found, 100 shown here

  1. ncbi Intraflagellar transport
    Joel L Rosenbaum
    Department of Molecular, Cellular and Developmental Biology, Yale University, New Haven, Connecticut 06520, USA
    Nat Rev Mol Cell Biol 3:813-25. 2002
    ..Recent results indicate that defects in IFT might be a primary cause of some human diseases...
  2. pmc MKS and NPHP modules cooperate to establish basal body/transition zone membrane associations and ciliary gate function during ciliogenesis
    Corey L Williams
    Department of Cell Biology, University of Alabama, Birmingham, AL 35294, USA
    J Cell Biol 192:1023-41. 2011
    ....
  3. pmc Polycystic kidney disease
    Peter C Harris
    Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota 55905, USA
    Annu Rev Med 60:321-37. 2009
    ..Rare, syndromic forms of PKD also include defects of the eye, central nervous system, digits, and/or neural tube and highlight the role of cilia and pathways such as Wnt and Hh in their pathogenesis...
  4. ncbi The primary cilium as the cell's antenna: signaling at a sensory organelle
    Veena Singla
    Program in Developmental and Stem Cell Biology, and Diabetes Center, University of California, San Francisco, CA 94143 0525, USA
    Science 313:629-33. 2006
    ....
  5. pmc Cyclic AMP-mediated cyst expansion
    Darren P Wallace
    Department of Internal Medicine, University of Kansas Medical Center, Kansas City, KS, USA
    Biochim Biophys Acta 1812:1291-300. 2011
    ..This article is part of a Special Issue entitled: Polycystic Kidney Disease...
  6. ncbi Polycystic kidney disease
    Patricia 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
  7. pmc Systems biology approach to identify transcriptome reprogramming and candidate microRNA targets during the progression of polycystic kidney disease
    Priyanka Pandey
    Department of Medicine, Children s Hospital Boston Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA
    BMC Syst Biol 5:56. 2011
    ..miRNAs are small, regulatory non-coding RNAs, implicated in a wide spectrum of biological processes. Their expression levels are altered in several diseases including kidney cancer, diabetic nephropathy and PKD...
  8. ncbi Epithelial-to-mesenchymal transition in cyst lining epithelial cells in an orthologous PCK rat model of autosomal-recessive polycystic kidney disease
    Hiroko Togawa
    Department of Pediatrics, Wakayama Medical University, Wakayama, Japan
    Am J Physiol Renal Physiol 300:F511-20. 2011
    ..Our study clarified the nephron segment-specific cyst profile related to EMT in PCK rats. EMT may play a key role in polycystic kidney disease...
  9. pmc The mTOR pathway is regulated by polycystin-1, and its inhibition reverses renal cystogenesis in polycystic kidney disease
    Jonathan M Shillingford
    Department of Molecular, Cellular, and Developmental Biology, University of California, Santa Barbara, CA 93106 9610, USA
    Proc Natl Acad Sci U S A 103:5466-71. 2006
    ..These results indicate that PC1 has an important function in the regulation of the mTOR pathway and that this pathway provides a target for medical therapy of ADPKD...
  10. ncbi Polycystic kidney disease and therapeutic approaches
    Eun Young Park
    Department of Biological Science, Sookmyung Women s University, Seoul 140 742, Korea
    BMB Rep 44:359-68. 2011
    ..In this review, we will discuss recent approaches to PKD therapy. It provides important information regarding potential targets for PKD...
  11. pmc A transcriptional network in polycystic kidney disease
    Lionel Gresh
    Unité Expression Génétique et Maladies CNRS URA 1644, Departement de Biologie du Developpement, Institut Pasteur, Paris, France
    EMBO J 23:1657-68. 2004
    ..This may explain the increased proliferation of cystic cells in MODY5 patients carrying autosomal dominant mutations in HNF1beta...
  12. pmc Caspase inhibition reduces tubular apoptosis and proliferation and slows disease progression in polycystic kidney disease
    Yunxia Tao
    Division of Renal Diseases and Hypertension, University of Colorado Health Sciences Center, Renal Box C281, 4200 East 9th Avenue, Denver, CO 80262, USA
    Proc Natl Acad Sci U S A 102:6954-9. 2005
    ....
  13. ncbi A genetic screen in zebrafish identifies cilia genes as a principal cause of cystic kidney
    Zhaoxia Sun
    MIT, Center for Cancer Research and Biology Department, Building E17 Room 340, 77 Massachusetts Avenue, Cambridge, MA 02139, USA
    Development 131:4085-93. 2004
    ..Our results also suggest that lesions in genes involved in cilia formation and function are the predominant cause of cystic kidney disease, and that the genes identified here are excellent candidates for novel human PKD genes...
  14. ncbi Putative roles of cilia in polycystic kidney disease
    Paul Winyard
    UCL Institute of Child Health, London, WC1N 1EH, UK
    Biochim Biophys Acta 1812:1256-62. 2011
    ..Understanding how cilia fit into the other aspects of polycystic kidney disease biology is the challenge for the next decade. This article is part of a Special Issue entitled: Polycystic Kidney Disease...
  15. pmc The RNA-binding protein bicaudal C regulates polycystin 2 in the kidney by antagonizing miR-17 activity
    Uyen Tran
    Department of Cell Biology and Anatomy, LSU Health Sciences Center, MEB 6A12, 1901 Perdido Street, New Orleans, LA 70112, USA
    Development 137:1107-16. 2010
    ..Based on these data, we propose that the kidney phenotype in Bicc1(-/-) mutant mice is caused by dysregulation of a microRNA-based translational control mechanism...
  16. pmc Increased expression of secreted frizzled-related protein 4 in polycystic kidneys
    Daniel Romaker
    Renal Division, University Hospital Freiburg, Hugstetter Strasse 55, D 79106 Freiburg, Germany
    J Am Soc Nephrol 20:48-56. 2009
    ..Taken together, these observations suggest a potential role for SFRP4 in the pathogenesis of ADPKD...
  17. ncbi Animal models for human polycystic kidney disease
    Shizuko Nagao
    Education and Research Center of Animal Models for Human Diseases, Fujita Health University, 1 98 Dengakugakubo, Kutsukake cho, Toyoake, Aichi 470 1192, Japan
    Exp Anim 61:477-88. 2012
    ..Therefore, experimental animal models are indispensable for investigating molecular mechanisms of PKD onset and progression as well as potential therapeutic treatments...
  18. pmc Celecoxib inhibits growth of human autosomal dominant polycystic kidney cyst-lining epithelial cells through the VEGF/Raf/MAPK/ERK signaling pathway
    Tao Xu
    Department of Nephrology, Shanghai Jiao Tong University Affiliated Sixth People s Hospital, Shanghai 200233, China
    Mol Biol Rep 39:7743-53. 2012
    ..CXB can inhibit proliferation, suppress cell cycle progression, and induce apoptosis in ADPKD cyst-lining epithelial cells through the inhibition of the VEGF/VEGFR-2/Raf-1/MAPK/ERK signaling pathway...
  19. ncbi Modifier genes play a significant role in the phenotypic expression of PKD1
    Pamela R Fain
    Barbara Davis Center for Childhood Diabetes, University of Colorado Health Sciences Center, Denver, Colorado 80262, USA
    Kidney Int 67:1256-67. 2005
    ..There are significant familial phenotype differences; but it is not clear if this is due to differences in PKD1 mutations, differences in genetic background, or both...
  20. ncbi Overexpression of innate immune response genes in a model of recessive polycystic kidney disease
    M Mrug
    Division of Genetic and Translational Medicine, Department of Medicine, The University of Alabama at Birmingham, 720 20th Street South, Birmingham, AL 35294, USA
    Kidney Int 73:63-76. 2008
    ..We also found elevated complement component 3 activation in two other mouse-recessive models and human-recessive PKD. Our results suggest that abnormal complement component 3 activation is a key element of progression in PKD...
  21. pmc Disruption of a ciliary B9 protein complex causes Meckel syndrome
    William E Dowdle
    Department of Biochemistry and Biophysics, Cardiovascular Research Institute, University of California, San Francisco, 94158, USA
    Am J Hum Genet 89:94-110. 2011
    ..Our data indicate that B9d1 is required for normal Hh signaling, ciliogenesis, and ciliary protein localization and that B9d1 and B9d2 are essential components of a B9 protein complex, disruption of which causes MKS...
  22. ncbi Calcium restores a normal proliferation phenotype in human polycystic kidney disease epithelial cells
    Tamio Yamaguchi
    Kidney Institute, Department of Medicine, University of Kansas Medical Center, 3901 Rainbow Boulevard, MSN 3018, Kansas City, KS 66160, USA
    J Am Soc Nephrol 17:178-87. 2006
    ..Thus, increases in [Ca2+]i are able to restore the normal anti-mitogenic response to cAMP in cells that are derived from two genetically distinct forms of PKD...
  23. pmc A genetic interaction network of five genes for human polycystic kidney and liver diseases defines polycystin-1 as the central determinant of cyst formation
    Sorin V Fedeles
    Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA
    Nat Genet 43:639-47. 2011
    ....
  24. pmc Loss of oriented cell division does not initiate cyst formation
    Saori Nishio
    Section of Nephrology, Yale University School of Medicine, P O Box 208029, 333 Cedar Street, New Haven, CT 06520 8029, USA
    J Am Soc Nephrol 21:295-302. 2010
    ..In conclusion, loss of oriented cell division is a feature of Pkhd1 mutation and cyst formation, but it is neither sufficient to produce kidney cysts nor required to initiate cyst formation after mutation in Pkd1 or Pkd2...
  25. ncbi Polycystin-2 is a novel cation channel implicated in defective intracellular Ca(2+) homeostasis in polycystic kidney disease
    P M Vassilev
    Renal Division, Brigham and Women's Hospital, Boston, Massachusetts, 02115, USA
    Biochem Biophys Res Commun 282:341-50. 2001
    ..The dysfunction of such mutants may result in defective coupling of PC2 to intracellular Ca(2+) homeostasis associated with the pathogenesis of ADPKD...
  26. ncbi Calcium, cyclic AMP, and MAP kinases: dysregulation in polycystic kidney disease
    B D Cowley
    University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA
    Kidney Int 73:251-3. 2008
    ..Data regarding use of CCBs in human polycystic kidney disease (PKD) are limited and mixed. Thus, it is premature to extrapolate these findings to human PKD...
  27. pmc A mitotic transcriptional switch in polycystic kidney disease
    Francisco Verdeguer
    Gene Expression, Development and Disease Laboratory, Developmental Biology Department, Institut Pasteur, Paris, France
    Nat Med 16:106-10. 2010
    ..This association suggests that HNF-1beta is a bookmarking factor that is necessary for reopening the chromatin of target genes after mitotic silencing...
  28. ncbi Inhibition of mTOR with sirolimus does not attenuate progression of liver and kidney disease in PCK rats
    Catharina Renken
    Department of Pediatrics, University Children s Hospital, Rostock, Rostock, Germany
    Nephrol Dial Transplant 26:92-100. 2011
    ..We examined the effects of sirolimus in PCK rats, an orthologous animal model of human ARPKD...
  29. pmc Polycystic kidney diseases: from molecular discoveries to targeted therapeutic strategies
    O Ibraghimov-Beskrovnaya
    Cell Biology, Genzyme Corporation, 5 Mountain Road, Framingham, MA 01701 9322, USA
    Cell Mol Life Sci 65:605-19. 2008
    b>Polycystic kidney diseases (PKDs) represent a large group of progressive renal disorders characterized by the development of renal cysts leading to end-stage renal disease...
  30. pmc Hypoxia-inducible factor-1α (HIF-1α) and autophagy in polycystic kidney disease (PKD)
    Franck Belibi
    Division of Renal Diseases and Hypertension, University of Colorado at Denver and Health Sciences Center, Aurora, 80262, USA
    Am J Physiol Renal Physiol 300:F1235-43. 2011
    ..The first demonstration of autophagosomes in PKD kidneys is reported. Inhibition of HIF-1α did not have a therapeutic effect...
  31. ncbi bcl-2 deficiency in mice leads to pleiotropic abnormalities: accelerated lymphoid cell death in thymus and spleen, polycystic kidney, hair hypopigmentation, and distorted small intestine
    S Kamada
    Department of Medical Genetics, Biomedical Research Center, Osaka University Medical School, Yamadaoka, Japan
    Cancer Res 55:354-9. 1995
    ..Our bcl-2-/- mice also revealed novel defects in the small intestine, characterized by retarded development, accelerated exfoliation of epithelial cells, and very few mitotic progenitor cells...
  32. ncbi Polycystic kidney disease prevented by transgenic RNA interference
    P Bouillet
    Cell Death Differ 12:831-3. 2005
  33. ncbi Molecular genetics of polycystic kidney disease
    J P Calvet
    Department of Biochemistry and Molecular Biology, University of Kansas Medical Center, Kansas City, USA
    J Nephrol 11:24-34. 1998
    ....
  34. ncbi Increased water intake decreases progression of polycystic kidney disease in the PCK rat
    Shizuko Nagao
    Education and Research Center of Animal Models for Human Diseases, Fujita Health University, Toyoake, Aichi, Japan
    J Am Soc Nephrol 17:2220-7. 2006
    ..It is suggested that limiting serum AVP levels by increased water intake may be beneficial to some patients with PKD...
  35. ncbi Murine congenital polycystic kidney disease: a model for studying development of cystic disease
    G M Preminger
    J Urol 127:556-60. 1982
  36. ncbi Survival after end-stage renal disease in autosomal dominant polycystic kidney disease: contribution of extrarenal complications to mortality
    R D Perrone
    Divisions of Nephrology and Clinical Care Research, Department of Medicine, New England Medical Center and Tufts University School of Medicine, Boston, MA 02111, USA
    Am J Kidney Dis 38:777-84. 2001
    ..Mortality secondary to extrarenal complications was substantially lower than that secondary to cardiovascular or cerebrovascular disease...
  37. ncbi Apico-basal polarity in polycystic kidney disease epithelia
    Patricia D Wilson
    UCL Medical School, Royal Free Hospital, London, UK
    Biochim Biophys Acta 1812:1239-48. 2011
    ..This article is part of a Special Issue entitled: Polycystic Kidney Disease...
  38. ncbi Juvenile cystic kidneys (jck): a new mouse mutation which causes polycystic kidneys
    A Atala
    Division of Urology, Children s Hospital, Boston, Massachusetts
    Kidney Int 43:1081-5. 1993
    ..The study of these five mutations and their interactions should prove useful for understanding the mechanisms required to maintain the normal integrity of renal tubules...
  39. ncbi What is the role of tubular epithelial cell apoptosis in polycystic kidney disease (PKD)?
    Charles L Edelstein
    Division of Renal Diseases and Hypertension, University of Colorado School of Medicine, Denver, Colorado 80262, USA
    Cell Cycle 4:1550-4. 2005
    ..Thus, there is evidence that both epithelial cell apoptosis and proliferation are dysregulated in ADPKD and may represent a general mechanism for cyst growth...
  40. ncbi Matrix metalloproteinase-2 in a murine model of infantile-type polycystic kidney disease
    C A Rankin
    Department of Biochemistry and Molecular Biology, University of Kansas Medical Center, Kansas City 66160 7400, USA
    J Am Soc Nephrol 10:210-7. 1999
    ..MMP-2 was abnormally localized to the interstitium and to foci between cysts, suggesting that MMP-2 may regulate collagen accumulation at those sites, thus allowing cyst enlargement and limiting the severity of interstitial fibrosis...
  41. ncbi Cystic disease of the kidney
    Patricia 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
    ....
  42. ncbi Defective planar cell polarity in polycystic kidney disease
    Evelyne Fischer
    Gene Expression and Disease Unit, Centre National de la Recherche Scientifique CNRS FRE 2850, Pasteur Institute, 25 rue du Docteur Roux, 75724 Paris Cedex 15, France
    Nat Genet 38:21-3. 2006
    ..These results suggest that oriented cell division dictates the maintenance of constant tubule diameter during tubular lengthening and that defects in this process trigger renal tubular enlargement and cyst formation...
  43. pmc The Oak Ridge Polycystic Kidney mouse: modeling ciliopathies of mice and men
    Jonathan M Lehman
    Department of Cell Biology, University of Alabama at Birmingham, Birmingham, Alabama 35294, USA
    Dev Dyn 237:1960-71. 2008
    ....
  44. pmc Microarray-based approach identifies microRNAs and their target functional patterns in polycystic kidney disease
    Priyanka Pandey
    Medical Research Center, University Hospital Mannheim, Mannheim, Germany
    BMC Genomics 9:624. 2008
    ..Although involvement of certain genes and transcriptional networks in PKD development has been shown, not much is known how they are regulated molecularly...
  45. ncbi Gene profiling of polycystic kidneys
    Gisela Schieren
    Renal Division, Department of Internal Medicine, Ruhr University Hospital Bochum at Marienhospital Herne, Freiburg, Germany
    Nephrol Dial Transplant 21:1816-24. 2006
    ..Fluid secretion with cyst expansion and reactive changes in the extracellular matrix composition combined with increased apoptosis and proliferation rates have been implicated in cystogenesis...
  46. pmc Involvement of hypoxia-inducible transcription factors in polycystic kidney disease
    Wanja Michael Bernhardt
    Department of Nephrology and Hypertension, Friedrich Alexander University, Krankenhausstrasse 12, D 91054 Erlangen, Germany
    Am J Pathol 170:830-42. 2007
    ..In conclusion, HIF accumulation in human and rat PKD seems to be responsible for increased EPO production and pericystic hypervascularity and may have an impact on progression of PKD...
  47. ncbi Bcl-2-deficient mice demonstrate fulminant lymphoid apoptosis, polycystic kidneys, and hypopigmented hair
    D J Veis
    Department of Medicine, Howard Hughes Medical Institute, Washington University School of Medicine, St Louis, Missouri 63110
    Cell 75:229-40. 1993
    ..The abnormalities in these loss of function mice argue that Bcl-2 is a death repressor molecule functioning in an antioxidant pathway...
  48. ncbi Calcium signaling and polycystin-2
    Georgia I Anyatonwu
    Department of Pharmacology, Yale University School of Medicine, New Haven, CT, USA
    Biochem Biophys Res Commun 322:1364-73. 2004
    ..Elucidation of the signaling pathway involved in normal PC1/PC2 function, the functional consequences of PC1/PC2 mutation, and the role of Ca2+ signaling will all help to unravel the molecular mechanisms of cystogenesis in PKD...
  49. pmc Triptolide is a traditional Chinese medicine-derived inhibitor of polycystic kidney disease
    Stephanie J Leuenroth
    Department of Molecular, Cellular, and Developmental Biology, Yale University, New Haven, CT 06511, USA
    Proc Natl Acad Sci U S A 104:4389-94. 2007
    ..We anticipate that small molecule induction of PC2-dependent calcium release is likely to be a valid therapeutic strategy for ADPKD...
  50. ncbi Polycystic kidney disease: genes, proteins, animal models, disease mechanisms and therapeutic opportunities
    V E Torres
    Division of Nephrology and Hypertension, Mayo College of Medicine, Rochester, MN 55905, USA
    J Intern Med 261:17-31. 2007
    ..This review summarizes the rationale for these treatments, the results of preclinical trials and the prospects for clinical trials, some already in early phases of implementation...
  51. ncbi Polycystins: from mechanosensation to gene regulation
    Patrick Delmas
    Laboratoire de Neurophysiologie Cellulaire, CNRS UMR 6150, Faculte de Medecine, IFR Jean Roche, Bd Pierre Dramard, 13916 Marseille Cedex 20, France
    Cell 118:145-8. 2004
    ..This review focuses on the pros and cons of their candidacy as mechanically gated channels and on recent findings that have significantly advanced our physiological insight...
  52. ncbi Curcumin inhibits renal cyst formation and enlargement in vitro by regulating intracellular signaling pathways
    Jinsheng Gao
    Department of Pharmacology, School of Basic Medical Sciences, Peking University, 38 Xueyuan Lu, Haidian, Beijing, China
    Eur J Pharmacol 654:92-9. 2011
    ..These results define that curcumin inhibits renal cyst formation and enlargement and suggest that curcumin might be developed as a candidate drug for polycystic kidney disease...
  53. ncbi The mammalian Nek1 kinase is involved in primary cilium formation
    Ohad Shalom
    The Mina and Everard Goodman, Faculty of Life Sciences, Bar Ilan University, Ramat Gan 52900, Israel
    FEBS Lett 582:1465-70. 2008
    Recent studies implicate primary cilium (PC) proteins in the etiologies of various polycystic kidney diseases (PKD). NIMA-related kinases (NRKs) are conserved serine/threonine kinases, which are usually defined as 'mitotic kinases'...
  54. ncbi Cystic renal neoplasia following conditional inactivation of apc in mouse renal tubular epithelium
    Chao Nan Qian
    Laboratories of Cancer Genetics and Cell Signaling and Carcinogenesis, Van Andel Research Institute, Grand Rapids, Michigan 49508, USA
    J Biol Chem 280:3938-45. 2005
    ..Our results confirm an important role for proper regulation of Wnt/beta-catenin signaling in renal development and provide evidence that dysregulation of the pathway can initiate tumorigenesis in the kidney...
  55. ncbi Early embryonic renal tubules of wild-type and polycystic kidney disease kidneys respond to cAMP stimulation with cystic fibrosis transmembrane conductance regulator/Na(+),K(+),2Cl(-) Co-transporter-dependent cystic dilation
    Brenda S Magenheimer
    Department of Biochemistry and Molecular Biology, University of Kansas Medical Center, Kansas City, KS 66160, USA
    J Am Soc Nephrol 17:3424-37. 2006
    ....
  56. pmc Mouse models of polycystic kidney disease induced by defects of ciliary proteins
    Je Yeong Ko
    Department of Biological Science, Sookmyung Women s University, Seoul 140 742, Korea
    BMB Rep 46:73-9. 2013
    ..In this review, we will describe the general role of cilia in renal epithelial cells, and the relationship between ciliary defects and PKD. We also discuss mouse models of PKD related to ciliary defects based on recent studies...
  57. ncbi Attenuated expression of epithelial cell adhesion molecules in murine polycystic kidney disease
    M V Rocco
    Department of Medicine, University of Pennsylvania, Philadelphia 19104
    Am J Physiol 262:F679-86. 1992
    ....
  58. pmc MicroRNAs regulate renal tubule maturation through modulation of Pkd1
    Vishal Patel
    Department of Internal Medicine Nephrology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, MC 8856, Dallas, TX 75390, USA
    J Am Soc Nephrol 23:1941-8. 2012
    ..In conclusion, miRNAs are essential for the maturation of renal tubules, and Pkd1 is a target of miR-200. These results also suggest that miRNAs may modulate PKD1 gene dosage and play a role in the initiation of cystogenesis...
  59. pmc Elevated c-myc protooncogene expression in autosomal recessive polycystic kidney disease
    B D Cowley
    Department of Medicine, University of Kansas Medical Center, Kansas City 66103
    Proc Natl Acad Sci U S A 84:8394-8. 1987
    The polycystic kidney diseases (PKDs) are a group of disorders characterized by the growth of epithelial cysts from the nephrons and collecting ducts of kidney tubules...
  60. pmc Genetic identification of two major modifier loci of polycystic kidney disease progression in pcy mice
    D D Woo
    Department of Medicine, UCLA School of Medicine, Los Angeles, California 90095 1689, USA
    J Clin Invest 100:1934-40. 1997
    ..Characterization of these novel modifying loci may provide additional insights into the pathogenesis of polycystic kidney diseases.
  61. pmc Epidermal growth factor receptor activity mediates renal cyst formation in polycystic kidney disease
    W G Richards
    Life Sciences Division, Oak Ridge National Laboratory, Oak Ridge, Tennessee 37831, USA
    J Clin Invest 101:935-9. 1998
    ..These results suggest that changes in the expression of the EGFR contribute to the formation of cysts in the collecting ducts, and that drugs that target the tyrosine kinase activity of the EGFR may potentially be therapeutic in ARPKD...
  62. pmc Dose-dependent effects of sirolimus on mTOR signaling and polycystic kidney disease
    Zlata Novalic
    Department of Human and Clinical Genetics, Leiden University Medical Center, The Netherlands
    J Am Soc Nephrol 23:842-53. 2012
    ..Mechanisms to increase bioavailability or to target mTOR inhibitors more specifically to kidneys, alone or in combination with other compounds, may improve the potential for these therapies in PKD...
  63. ncbi Polycystic kidney disease at end-stage renal disease in the United States: patient characteristics and survival
    K C Abbott
    Nephrology Service, Walter Reed Army Medical Center, Washington, DC, 20307 5001, USA
    Clin Nephrol 57:208-14. 2002
    ..The patient characteristics and mortality associated with autosomal dominant polycystic kidney disease have not been characterized for a national sample of end-stage renal disease (ESRD) patients...
  64. pmc Genetic localization of interacting modifiers affecting severity in a murine model of polycystic kidney disease
    S Kuida
    Genetics Division, Brigham and Women s Hospital, Harvard Medical School, Boston, Massachusetts 02115 USA
    Genome Res 10:49-54. 2000
    ....
  65. ncbi Milder presentation of recessive polycystic kidney disease requires presence of amino acid substitution mutations
    Laszlo Furu
    Departments of Internal Medicine and Genetics, Yale University School of Medicine, New Haven, Connecticut, USA
    J Am Soc Nephrol 14:2004-14. 2003
    ..The nature of the germline mutations in ARPKD plays a significant role in determining clinical outcome...
  66. pmc Inhibition of glucosylceramide accumulation results in effective blockade of polycystic kidney disease in mouse models
    Thomas A Natoli
    Department of Cell Biology, Genzyme Corporation, Framingham, Massachusetts, USA
    Nat Med 16:788-92. 2010
    ..Taken together, our data suggest that inhibition of GlcCer synthesis represents a new and effective treatment option for PKD...
  67. pmc Meckelin is necessary for photoreceptor intraciliary transport and outer segment morphogenesis
    Gayle B Collin
    Jackson Laboratory, 600 Main Street, Bar Harbor, ME 04609, USA
    Invest Ophthalmol Vis Sci 53:967-74. 2012
    ..In this study, the retinal morphology and ciliary function in a mouse model for Meckel Syndrome Type 3 (MKS3) throughout the course of photoreceptor development was examined...
  68. pmc Signal transducer and activator of transcription-6 (STAT6) inhibition suppresses renal cyst growth in polycystic kidney disease
    Erin E Olsan
    Department of Molecular, Cellular and Developmental Biology and Neuroscience Research Institute, University of California, Santa Barbara, CA 93106, USA
    Proc Natl Acad Sci U S A 108:18067-72. 2011
    ..Treatment of PKD mice with this drug leads to amelioration of the renal cystic disease similar to genetic STAT6 inactivation. These results suggest STAT6 as a promising drug target for treatment of ADPKD...
  69. ncbi Quantitative trait loci modulate renal cystic disease severity in the mouse bpk model
    L M Guay-Woodford
    Department of Medicine, University of Alabama at Birmingham, 35294, USA
    J Am Soc Nephrol 11:1253-60. 2000
    ..It is hypothesized that the gene encoding the polycystin-binding partner RGS7 is a candidate for the Chr 1 genetic modifier...
  70. ncbi Altered extracellular matrix component gene expression in murine polycystic kidney
    I Ebihara
    Department of Medicine, Juntendo University School of Medicine, Tokyo, Japan
    Ren Physiol Biochem 18:73-80. 1995
    ..These data suggest that changes in the expression of basement membrane components and interstitial collagens are associated with the development of polycystic kidney disease...
  71. ncbi Expression of matrix metalloproteinase in the fluids of renal cystic lesions
    Hiroshi Harada
    Division of Pathophysiological Science, Department of Pathology Pathophysiology, Hokkaido University Graduate School of Medicine, Sapporo, Japan
    J Urol 168:19-22. 2002
    ..We analyzed the expression of matrix metalloproteinase (MMP) in the fluids of benign and malignant renal cystic lesions to clarify matriolytic activities in the cyst...
  72. ncbi Alterations in renal cytosolic phospholipase A2 and cyclooxygenases in polycystic kidney disease
    Harold M Aukema
    Department of Human Nutritional Sciences, University of Manitoba, Winnipeg, MB, Canada, R3T 2N2
    FASEB J 17:298-300. 2003
    ..The altered levels of these eicosanoid-regulating enzymes has implications for the use of NSAIDS and specific COX inhibitors in individuals with this disorder...
  73. ncbi Collecting duct carcinoma exhibiting diastase-resistant PAS-positive globular cytoplasmic inclusions and rhabdoid features arising in adult polycystic kidney disease: a case report
    Naoto Kuroda
    Department of Pathology, Kochi Medical School, Japan
    Int J Surg Pathol 12:171-7. 2004
    ..Immunohistochemically, large esosinophilic inclusions were reactive for vimentin. Although these findings are rare in CDCs, they should be recognized in the pathologic spectrum of CDCs. Int J Surg Pathol 12(2):171-177, 2004..
  74. ncbi VEGF receptor inhibition slows the progression of polycystic kidney disease
    Y Tao
    Department of Internal Medicine, Texas Tech University Health Sciences Center, Amarillo, Texas 79106, USA
    Kidney Int 72:1358-66. 2007
    ..Our studies show that inhibition of VEGF function may be an important therapeutic option to delay the progression of polycystic kidney disease...
  75. ncbi c-myc-induced apoptosis in polycystic kidney disease is independent of FasL/Fas interaction
    Martin Couillard
    Institut de Recherches Cliniques de Montreal, Faculte de Medecine de l Universite de Montreal, Montreal, Quebec, H2W 1R7 Canada
    Cancer Res 62:2210-4. 2002
    ..Our study proves that c-myc-induced apoptosis can be independent of the FasL/Fas pathway in vivo and implicates the existence of a novel c-myc-driven apoptotic pathway...
  76. ncbi Genetic analysis of a quantitative trait in a mouse model of polycystic kidney disease
    O A Iakoubova
    Genetics Division, Brigham and Women s Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA
    Am J Respir Crit Care Med 156:S72-7. 1997
    ..This type of effect, which is an example of genetic epistasis, will make the molecular characterization of loci that contribute to complex traits markedly more difficult than the analysis of monogenic disorders...
  77. ncbi Comparison of Pkd1-targeted mutants reveals that loss of polycystin-1 causes cystogenesis and bone defects
    W Lu
    Renal Division, Department of Medicine, Brigham and Women s Hospital, Boston, MA 02115, USA
    Hum Mol Genet 10:2385-96. 2001
    ..Our data demonstrate for the first time that loss of polycystin-1 leads to cyst formation and defective skeletogenesis, and indicate that polycystin-1 is critical in both epithelium and chondrocyte development...
  78. ncbi Calcium restriction allows cAMP activation of the B-Raf/ERK pathway, switching cells to a cAMP-dependent growth-stimulated phenotype
    Tamio Yamaguchi
    Department of Biochemistry, The Kidney Institute, University of Kansas Medical Center, Kansas City, Kansas 66160, USA
    J Biol Chem 279:40419-30. 2004
    ..We conclude that disruption of calcium mobilization in cells that are normally growth-inhibited by cAMP can derepress the B-Raf/ERK pathway, thus converting these cells to a phenotype that is growth-stimulated by cAMP...
  79. ncbi Long-lasting arrest of murine polycystic kidney disease with CDK inhibitor roscovitine
    Nikolay O Bukanov
    Cell Biology, Genzyme Corporation, 5 Mountain Road, Framingham, Massachusetts 01701 9322, USA
    Nature 444:949-52. 2006
    b>Polycystic kidney diseases (PKDs) are primarily characterized by the growth of fluid-filled cysts in renal tubules leading to end-stage renal disease...
  80. ncbi NIMA-related kinases defective in murine models of polycystic kidney diseases localize to primary cilia and centrosomes
    Moe R Mahjoub
    Department of Molecular Biology and Biochemistry, Simon Fraser University, Burnaby, British Columbia, Canada
    J Am Soc Nephrol 16:3485-9. 2005
    A key feature of the polycystic kidney diseases is aberrant cell proliferation, a consequence of dysfunctional ciliary signaling...
  81. pmc Kidney-specific inactivation of the KIF3A subunit of kinesin-II inhibits renal ciliogenesis and produces polycystic kidney disease
    Fangming Lin
    Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
    Proc Natl Acad Sci U S A 100:5286-91. 2003
    ..Most generally, the phenotype of Kif3a mutant mice suggests a role for primary cilia in the maintenance of lumen-forming epithelial differentiation...
  82. ncbi A polycystic kidney-disease gene homologue required for male mating behaviour in C. elegans
    M M Barr
    Howard Hughes Medical Institute and Division of Biology, California Institute of Technology, Pasadena 91125, USA
    Nature 401:386-9. 1999
    ..PKD-2, the C. elegans homologue of PKD2, is localized to the same neurons as LOV-1, suggesting that they function in the same pathway...
  83. ncbi Early development of polycystic kidney disease in transgenic mice expressing an activated mutant of the beta-catenin gene
    S Saadi-Kheddouci
    INSERM U129, ICGM, 24 rue du Faubourg St Jacques, 75014 Paris, France
    Oncogene 20:5972-81. 2001
    ....
  84. pmc Advances in the pathogenesis and treatment of polycystic kidney disease
    Vishal Patel
    Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
    Curr Opin Nephrol Hypertens 18:99-106. 2009
    ..Mouse models of PKD, especially those with mutations in genes that are orthologous to human disease genes, have provided insights into the pathogenesis of cyst formation and advanced the preclinical testing of new drugs...
  85. ncbi Kidney development and disease in the zebrafish
    Iain A Drummond
    Department of Medicine, Harvard Medical School and Renal Unit, Massachusetts General Hospital, 149 13th Street, Charlestown, MA 02129, USA
    J Am Soc Nephrol 16:299-304. 2005
    ..This short review outlines recent progress in applying the zebrafish pronephros to issues of human health and development...
  86. pmc Calcimimetic inhibits late-stage cyst growth in ADPKD
    Vincent H Gattone
    Department of Anatomy and Cell Biology, Indiana University School of Medicine, 635 Barnhill Drive, Indianapolis, Indiana 46202, USA
    J Am Soc Nephrol 20:1527-32. 2009
    ..The benefit of R-568 alone suggests calcium-sensing receptor modulation may have additional inhibitory effects on late-stage cyst growth resulting from a direct modulation of intracellular calcium...
  87. ncbi Identification of proteins that interact with the central coiled-coil region of the human protein kinase NEK1
    Marcelo J Surpili
    Centro de Biologia Molecular Estrutural CEBIME, Laboratorio Nacional de Luz Sincrotron LNLS, Rua Giuseppe Máximo Scolfaro 10 000, CP 6192, 13084 971 Campinas, SP, Brazil
    Biochemistry 42:15369-76. 2003
    ..Our results give further support to the previous observation that NEK1 is of functional importance for the etiology of PKD...
  88. ncbi [Polycystic kidney diseases: molecular genetics and counselling]
    James Lespinasse
    Division de génétique médicale, Hopital Sainte Justine, Montreal, Quebec, Canada
    Nephrol Ther 2:120-6. 2006
    ..It permits to determine the type of transmission, to describe the course and the major complications of the disease and to explain currents therapeutics possibilities...
  89. ncbi Therapeutic potential of cystic fibrosis transmembrane conductance regulator (CFTR) inhibitors in polycystic kidney disease
    Hongyu Li
    Department of Physiology and Pharmacology, University of Bristol, School of Medical Sciences, Bristol, UK
    BioDrugs 23:203-16. 2009
    ..The demonstration that CFTR inhibitors retard cyst expansion and kidney enlargement in mouse models of ADPKD provides proof of concept for the use of small-molecule CFTR inhibitors in the treatment of ADPKD...
  90. pmc Kidney-targeted Birt-Hogg-Dube gene inactivation in a mouse model: Erk1/2 and Akt-mTOR activation, cell hyperproliferation, and polycystic kidneys
    Masaya Baba
    Urologic Oncology Branch, National Cancer Institute Frederick, Frederick, MD 21702, USA
    J Natl Cancer Inst 100:140-54. 2008
    ..BHD encodes folliculin, a protein that may interact with the energy- and nutrient-sensing 5'-AMP-activated protein kinase-mammalian target of rapamycin (AMPK-mTOR) signaling pathways...
  91. ncbi Localization of Inv in a distinctive intraciliary compartment requires the C-terminal ninein-homolog-containing region
    Dai Shiba
    Department of Anatomy and Developmental Biology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
    J Cell Sci 122:44-54. 2009
    ....
  92. ncbi Role of primary cilia in the pathogenesis of polycystic kidney disease
    Bradley K Yoder
    Department of Cell Biology, University of Alabama at Birmingham, Birmingham, Alabama, USA
    J Am Soc Nephrol 18:1381-8. 2007
    ....
  93. ncbi Wnt signaling in polycystic kidney disease
    Thomas Benzing
    Renal Division, University Hospital Freiburg, Freiburg, Germany
    J Am Soc Nephrol 18:1389-98. 2007
    ..These spatial cues may be needed to position nascent tubules with a defined geometry...
  94. ncbi Polycystic kidney disease
    Peter Igarashi
    Division of Nephrology, Department of Internal Medicine, University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390 8856, USA
    J Am Soc Nephrol 18:1371-3. 2007
  95. ncbi Renal failure causes early death of bcl-2 deficient mice
    Lev M Fedorov
    Theodor Boveri Institut für Biowissenschaften, Biozentrum, Bayerische Julius Maximilians Universität, Am Hubland, 97074 Wurzburg, Germany
    Mech Ageing Dev 127:600-9. 2006
    ..Such models can be useful to study the influence of bcl-2 or other gene deficiency in individual organs (or tissues) on development and ageing of whole organism...
  96. ncbi Extracellular signal-regulated kinase inhibition slows disease progression in mice with polycystic kidney disease
    Sayu Omori
    Department of Pediatrics, Keio University School of Medicine, 35 Shinanomachi, Shinjuku ku, Tokyo 160 8582, Japan
    J Am Soc Nephrol 17:1604-14. 2006
    ..These results demonstrate that the expression of MAPK is dysregulated in cyst epithelium and that inhibition of ERK slowed the progression of renal disease in pcy mice...
  97. ncbi In vitro cystogenesis: the search for drugs antagonizing cyst development
    Oxana Ibraghimov-Beskrovnaya
    Genzyme Corporation, 5, Mountain Road, Framingham, MA 01701 9322, Etats Unis
    Nephrol Ther 2:S109-14. 2006
    ..We have used in vitro cystogenesis assay for a high throughput screen of small molecule drugs and identified drugs specifically inhibiting cystogenesis but not tubulogenesis in vitro...
  98. ncbi The roles of cilia in developmental disorders and disease
    Brent W Bisgrove
    Huntsman Cancer Institute Center for Children, Department of Oncological Sciences, University of Utah, Salt Lake City, UT 84112, USA
    Development 133:4131-43. 2006
    ..Here, we summarize an emerging view that in order to understand some complex developmental pathways and disease etiologies, one must consider the molecular functions performed by cilia...
  99. ncbi Cilia: tuning in to the cell's antenna
    Wallace F Marshall
    Department of Biochemistry and Biophysics, University of California San Francisco, 600 16th St, San Francisco, California 94143, USA
    Curr Biol 16:R604-14. 2006
    ..While these diseases highlight the pivotal roles of cilia in physiology and development, the mechanistic link between cilia, physiology, and disease remains unclear...
  100. ncbi Oral-facial-digital type I protein is required for primary cilia formation and left-right axis specification
    Maria Immacolata Ferrante
    Telethon Institute of Genetics and Medicine, Via P Castellino 111, 80131 Naples, Italy
    Nat Genet 38:112-7. 2006
    ....
  101. ncbi Genetics and pathogenesis of polycystic kidney disease
    Peter Igarashi
    Department of Internal Medicine, The University of Texas Southwestern Medical Center, Dallas, Texas, USA
    J Am Soc Nephrol 13:2384-98. 2002

Research Grants62

  1. FASEB SRC on
    Bradley K Yoder; Fiscal Year: 2013
    ..ALS), Senior-Loken Syndrome, Primary Ciliary Dyskinesia, Nephronophthisis, Retinitis Pigmentosa, and Polycystic Kidney Diseases (PKD). PKD itself affects 1 in 1,000 individuals ultimately requiring dialysis and transplantation...
  2. Genetic analysis of the ciliopathies ARPKD and Meckel syndrome
    Peter C Harris; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): In the last few years it has become clear that polycystic kidney diseases (PKDs) are ciliopathies, due to mutations in cilia related genes...
  3. FASEB on Biology of Cilia and Flagella
    Bradley K Yoder; Fiscal Year: 2010
    ..Ciliary Dyskinesia, Joubert Syndrome, Alstrom Syndrome, Retinitis Pigmentosa, Nephronophthisis, and Polycystic Kidney Diseases (PKD) are disorders of the primary cilium...
  4. Regulation of Genes Encoding Flagellar Proteins in Chlamydomonas reinhardtii
    JASON MITCHELL BROWN; Fiscal Year: 2010
    ..Many disorders, including polycystic kidney diseases and primary ciliary diskinesia, result from loss or malfunction of cilia...
  5. Novel role of AQP2 in cell migration and kidney tubular injury and repair
    HUA A LU; Fiscal Year: 2013
    ..of AQP2 has been frequently reported in a variety of transgenic animal models and in patients with polycystic kidney diseases with polyuria and coexisting structural and functional defects of the kidney tubules...
  6. The Role of Bicaudal-C in Polycystic Kidney Disease
    Oliver Wessely; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Polycystic Kidney Diseases (PKD) are the leading cause of end-stage renal failure and are characterized by the development of renal cysts along the entire length of the nephron...
  7. A forward genetic screen for PKD pathways in mice using the PiggyBac transposon
    Stefan Somlo; Fiscal Year: 2010
    ..A critical step for promoting deeper scientific understanding of the factors underlying polycystic kidney diseases and for promoting interest in efforts to develop therapies, is to achieve a compelling and precise ..
  8. FASEB SRC on "Polycystic Kidney Disease, From Bench to Bedside"
    Dorien J M Peters; Fiscal Year: 2011
    ..b>Polycystic kidney diseases are a group of inherited disorders that result in cyst development in the kidney often resulting in end ..
  9. Molecular Basis of Polarized Exocytosis
    Wei Guo; Fiscal Year: 2012
    ..of exocytosis, and contribute to our understanding of diseases such as neurological degeneration, polycystic kidney diseases, and cancer...
  10. Mechanisms of Polycystin and Cilia Function in ADPKD
    Stefan Somlo; Fiscal Year: 2013
    ..Last, First, Middle): Somlo, Stefan Project Summary/Abstract The functional connection between human polycystic kidney diseases and cilia is based in part on the overlap between genes whose mutation results in kidney cyst formation ..
  11. Center for Polycystic Kidney Disease Research at Yale
    Michael J Caplan; Fiscal Year: 2013
    ..at Yale is to facilitate translational research that will advance the understanding and treatment of polycystic kidney diseases. The Center will provide investigators both at Yale and across the country with access to highly ..
  12. CYSTIC DILATATION OF NEPHRONS IN TRANSGENIC INV MICE
    Carrie Phillips; Fiscal Year: 2005
    ..Inherited polycystic kidney diseases (PKD) of childhood include nephronophthisis, autosomal recessive polycystic kidney disease and medullary ..
  13. Xenopus Bicaudal-C a Model for Polycystic Kidney Disease
    Oliver Wessely; Fiscal Year: 2007
    b>Polycystic Kidney Diseases (PKD) are the leading cause of end-stage renal failure and require extensive treatments, such as dialysis and kidney transplantation...
  14. Characterization of the Murine pcy Mutation
    David Woo; Fiscal Year: 2005
    b>Polycystic kidney diseases (PKD) are among the leading causes of progressive renal failure in children and adults. Currently, there is no known therapy that can inhibit or retard the progression to renal failure in PKD patients...
  15. Molecular Genetics and Pathogenesis of ARPKD
    Guanqing Wu; Fiscal Year: 2004
    ..Taken together, the results of these studies will significantly advance our understanding of the pathogenesis of polycystic kidney diseases and the molecular mechanism of cyst formation and growth.
  16. MECHANISM OF EGF RECEPTOR MISSORTING IN PKD
    Sanjay Pimplikar; Fiscal Year: 1999
    ..In Polycystic Kidney Diseases, a group of disorders characterized by the progressive formation of renal cysts, several membrane ..
  17. Molecular networks: programming normal renal development and modeling disease pat
    Lisa Guay Woodford; Fiscal Year: 2007
    ..The meeting will be held in Pecs, Hungary from August 27-30, 2007, immediately preceding the 14th IPNA Congress in Budapest, Hungary (August 31- September 4, 2007). [unreadable] [unreadable] [unreadable]..
  18. Cystin, a lipid raft and cilia-associated protein in PKD
    Lisa Guay Woodford; Fiscal Year: 2008
    ..unreadable] [unreadable] [unreadable] [unreadable]..
  19. FASEB Conference -PKD Mechanisms and Clinical Impact
    Lisa Guay Woodford; Fiscal Year: 2005
    ..for the Federation of American Societies of Experimental Biology (FASEB) Summer Research Conference on "Polycystic Kidney Diseases: Molecular Pathways, Pathogenic Mechanisms and Translational Applications" to be held from August 6-11, ..
  20. Genetics and Pharmacogenetics in FSGS (PPG Project 4)
    Lisa Guay Woodford; Fiscal Year: 2007
    ..unreadable]..
  21. Novel Therapeutic Strategies for PKD
    Patricia Wilson; Fiscal Year: 2007
    b>Polycystic kidney diseases (PKD) affect greater than or equal too 500,000 patients in the US and > 6 million worldwide, but the only form of "therapy" is renal replacement by dialysis or transplantation...
  22. Cilial dysfunction and pathogenesis of obesity
    Bradley K Yoder; Fiscal Year: 2010
    ....
  23. REGULATION OF KIDNEY-SPECIFIC GENE EXPRESSION
    Peter Igarashi; Fiscal Year: 2010
    ..abstract_text> ..
  24. Pathogenesis of wpk-induced Renal and Cerebral Disease
    Vincent Gattone; Fiscal Year: 2008
    ..abstract_text> ..
  25. Pathogenesis of PKD in Mice Lacking Renal Cilia
    Peter Igarashi; Fiscal Year: 2008
    ....
  26. Roles of polycystin and calcium in B-Raf signaling
    DARREN WALLACE; Fiscal Year: 2004
    ..2) Determine the mechanism by which the overexpression of the C-terminal fragment of PC1 in M1 cells, a condition thought to disrupt PC1/PC2 function, alters cAMP-dependent B-Raf signaling. ..
  27. TG737 SIGNALING PATHWAY AND POLYCYSTIC KIDNEY
    Bradley Yoder; Fiscal Year: 2003
    ..Overall, results generated from this proposal will give important insight into the biological function of Tg737 and how disruption of the Tg737/polycystin-1 signaling pathway results in the formation of renal cyst. ..
  28. Transgene Induced HIV-Associated Nephropathy
    Vincent Gattone; Fiscal Year: 2004
    ..By studying this HIV-transgenic rat, we should be able to identify additional factors that contribute to the HIV-induced phenotype in GECs and provide new information that will lead to a rational treatment for HIVAN. ..
  29. Epithelial Cell Linage in the Kidney and Genitourinary Tract
    Peter Igarashi; Fiscal Year: 2007
    ....
  30. Conserved Mechanisms of Ciliogenesis
    Bradley Yoder; Fiscal Year: 2007
    ..abstract_text> ..
  31. TRANSCRIPTION FACTORS AND RENAL DEVELOPMENT
    Peter Igarashi; Fiscal Year: 2002
    ..Collectively, these studies should provide fundamental insights into transcriptional regulation of kidney morphogenesis and cystogenesis. ..
  32. High Pressure Freezing and Processing Unit
    Vincent Gattone; Fiscal Year: 2005
    ..The research and physical environment, EM Center personnel and IU School of Medicine institutional support make the IU EM Center an excellent place for the appropriate use of the Leica HPF and Processor System. ..
  33. MECHANISMS OF WATER TRANSPORT IN RENAL EPITHELIA
    Alan Verkman; Fiscal Year: 2008
    ..Methods will include fluorescence photobleaching recovery, energy transfer and ratio imaging. Also, specific water/solute transporting properties (Pd, c^)of the mammalian aquaporins will be measured. ..
  34. GENETICS OF AUTOSOMAL DOMINANT POLYCYSTIC LIVER DISEASE
    Stefan Somlo; Fiscal Year: 2010
    ..At the same time, we will improve our understanding of ADPLD as a disease condition and develop insights that will help patients affected by this condition. ..
  35. High-Content Cell-Based Screening for Modulators of Autophagy
    Xiao Ming Yin; Fiscal Year: 2007
    ..Our long term hope is that the knowledge and the materials developed through this study will be valuable for future clinical applications. [unreadable] [unreadable] [unreadable]..
  36. Intercellular Signaling in Obstructive Nephropathy
    ROBERT CHEVALIER; Fiscal Year: 2005
    ..This will lead to improved outcomes in adulthood. ..
  37. IMMUNOGENETICS OF TYPE 1 DIABETES IN A BEDOUIN FAMILY
    Pamela Fain; Fiscal Year: 2003
    ....
  38. Cux-1 and cell cycle regulation in kidney development
    Gregory Vanden Heuvel; Fiscal Year: 2009
    ..4. Define the relationship between Cux-1, TLE-4, and Notch signaling during podocyte development and nephrogenesis. These studies will provide novel insights into the mechanisms of cell proliferation during development. ..
  39. ICAM1 gene inactivation protects against ischemic injury
    Robert Montgomery; Fiscal Year: 2006
    ..This work has important implications for the selective inactivation of gene function in allografts through ex vivo gene transfer. ..
  40. STEM CELL THERAPY IN ACUTE RENAL FAILURE
    Fangming Lin; Fiscal Year: 2007
    ....
  41. Role of airway submucosal glands in CF lung disease
    Alan S Verkman; Fiscal Year: 2010
    ..The data will provide a rigorous scientific rationale for use of small-molecules in CF therapy, and establish widely useful assays to prioritize small molecules for clinical development. ..
  42. Opportunistic Oral HSV-- Mechanisms of Reactivation
    Craig Miller; Fiscal Year: 2006
    ....
  43. PATHOPHYSIOLOGY OF THE AGING KIDNEY
    Sharon Anderson; Fiscal Year: 2001
    ..The use of a combined physiological, biochemical, immunohistochemical, and molecular biologic approach should gain insight into this clinically important but understudied problem. ..
  44. Gene transfer to the kidney using lentiviral vectors
    Frank Park; Fiscal Year: 2004
    ..abstract_text> ..
  45. APPLICATION OF NOVEL OPTICAL METHODS TO CELL DYNAMICS
    Alan Verkman; Fiscal Year: 2001
    ..These studies will address the hypothesis that the membrane skeleton of these cells is a mobile, dynamic structure. ..
  46. TRANSPLANT IMMUNOLOGY OF IL2 IFNY DOUBLE KNOCKOUT MICE
    Martin Zand; Fiscal Year: 2002
    ..In contrast, if the DKO mice are tolerant to islet allografts, we will determine the mechanism by adoptive transfer of tolerance inducing T lymphocyte subsets. ..
  47. A NOVEL F BOX PROTEIN REGULATING MITOSIS
    Peter Jackson; Fiscal Year: 2004
    ..Understanding how SBP5 blocks mitosis and induces apoptosis may provide important insight for creating new cancer chemotherapeutic drugs that act similarly or synergistically with vinca alkaloids and Taxol. ..
  48. Towards renal regeneration
    Melissa Little; Fiscal Year: 2004
    ..Human ES cell work will be performed using ES01, 02, 03, 04, 05 & 06 listed on the NIH ES cell line registry. ..
  49. EPITHELIAL P2X PURINERGIC RECEPTOR CHANNELS
    Erik Schwiebert; Fiscal Year: 2003
    ..abstract_text> ..
  50. PHYSIOLOGY AND MOLECULAR BIOLOGY OF LUNG WATER TRANSPORT
    Alan Verkman; Fiscal Year: 2007
    ..abstract_text> ..
  51. Centriole Orientation During Left/Right Symmetry Breaking in the Mouse
    Wallace Marshall; Fiscal Year: 2007
    ..unreadable] [unreadable] [unreadable]..
  52. Polycystin-1, ATP-induced Ca2+ entry & Cl - conductance
    Michael Sutters; Fiscal Year: 2007
    ..The long-term aim of his work is to facilitate the design of new therapeutic strategies to control disease progression through delineation of the mechanism of cyst expansion in ADPKD ..
  53. Small Molecule Inhibitors of Cilia
    Wallace Marshall; Fiscal Year: 2007
    ..unreadable] [unreadable] [unreadable]..
  54. Multi-photon microscope for the Indiana Center for Biological Microscopy
    Kenneth Dunn; Fiscal Year: 2008
    ..unreadable] [unreadable] [unreadable]..
  55. APPLICATION OF NOVEL OPTICAL METHODS TO CELL DYNAMICS
    Alan Verkman; Fiscal Year: 2006
    ..abstract_text> ..
  56. Bc1-2 Family Proteins in the Ischemic Neuronal Injury
    Xiao Ming Yin; Fiscal Year: 2006
    ..To characterize the molecular interactions of Bid and Bax in inducing dysfunction of mitochondria isolated from the brain. ..
  57. Genetic & Clinical Risk for Human SLE Nephritis
    Lee Hebert; Fiscal Year: 2007
    ..Lay Summary: This research will lead to earlier diagnosis and treatment of kidney flares in lupus, and more effective use of available medications, resulting in improved clinical outcomes for SLE patients. ..
  58. Endocytic transport in cultured renal epithelia
    Kenneth Dunn; Fiscal Year: 2005
    ..abstract_text> ..
  59. PATHOPHYSIOLOGY OF DIABETIC NEPHROPATHY
    Sharon Anderson; Fiscal Year: 2007
    ..abstract_text> ..