Research Topics
Genomes and Genes | Oxana Ibraghimov-BeskrovnayaSummaryAffiliation: Genzyme Corporation Country: USA Publications
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Publications
In vitro cystogenesis: the search for drugs antagonizing cyst developmentOxana 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...
mTOR signaling in polycystic kidney diseaseOxana Ibraghimov-Beskrovnaya
Cell Biology, Genzyme Corporation, 49 New York Avenue, Framingham, MA, USA
Trends Mol Med 17:625-33. 2011..We discuss the mTOR pathway in depth, highlighting recent progress in understanding its role in PKD and the current results of clinical trials...
Polycystic kidney diseases: from molecular discoveries to targeted therapeutic strategiesO Ibraghimov-Beskrovnaya
Cell Biology, Genzyme Corporation, 5 Mountain Road, Framingham, MA 01701 9322, USA
Cell Mol Life Sci 65:605-19. 2008..We discuss available models and challenges for therapeutic discovery as well as summarize the results from preclinical experimental treatments targeting key disease-specific pathways...
Targeting dysregulated cell cycle and apoptosis for polycystic kidney disease therapyOxana Ibraghimov-Beskrovnaya
Genzyme Corporation, Framingham, Massachusetts 01701, USA
Cell Cycle 6:776-9. 2007..Thus, therapies directly targeting coordinate regulation of proliferation and apoptosis are emerging as effective approaches to treat multiple renal diseases...
Pkd1 and Nek8 mutations affect cell-cell adhesion and cilia in cysts formed in kidney organ culturesThomas A Natoli
Department of Cell Biology, Genzyme Corporation, 5 Mountain Rd, Framingham, MA 01701 9322, USA
Am J Physiol Renal Physiol 294:F73-83. 2008..Therefore, embryonic kidney organ culture represents a relevant model for studying molecular cystogenesis and a rapid tool for the screening for therapies that block cystic growth...
Functional polycystin-1 expression is developmentally regulated during epithelial morphogenesis in vitro: downregulation and loss of membrane localization during cystogenesisNikolay O Bukanov
Genzyme Corporation, 5 Mountain Road, Framingham, MA 01701 9322, USA
Hum Mol Genet 11:923-36. 2002..We suggest that the loss of polycystin-1 from its basolateral location in tubular epithelium may alter critical pathways controlling normal tubulogenesis leading to cystic transformation...
Development of polycystic kidney disease in juvenile cystic kidney mice: insights into pathogenesis, ciliary abnormalities, and common features with human diseaseLaurie A Smith
Genzyme Corporation, 5 Mountain Road, Framingham, MA 01701 9322, USA
J Am Soc Nephrol 17:2821-31. 2006..Collectively, these data demonstrate that the jck mice should be useful for testing potential therapies and for studying the molecular mechanisms that link ciliary structure/function and cystogenesis...
Inhibition of glucosylceramide accumulation results in effective blockade of polycystic kidney disease in mouse modelsThomas 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...
Loss of GM3 synthase gene, but not sphingosine kinase 1, is protective against murine nephronophthisis-related polycystic kidney diseaseThomas A Natoli
Department of Cell Biology, Genzyme Corporation, Framingham, MA 01701, USA
Hum Mol Genet 21:3397-407. 2012..Together, these data suggest that genes involved in the SL metabolism may be modifiers of cystogenesis, and suggest GM3 synthase as a new anti-cystic therapeutic target...
Serum and urinary biomarker signatures for rapid preclinical in vivo assessment of CDK inhibition as a therapeutic approach for PKDSarah Moreno
Cell Biology, Genzyme Corporation, Framingham, Massachusetts 01701 9322, USA
Cell Cycle 7:1856-64. 2008..This biomarker signature provides a necessary tool for further assessment of CDK inhibitors as therapeutic agents for PKD...
Canine PKD1 is a single-copy gene: genomic organization and comparative analysisWilliam R Dackowski
Genzyme Corporation, 5 Mountain Road, Framingham, Massachusetts, 01701 9322, USA
Genomics 80:105-12. 2002..These data enable detailed analysis of the role of polycystin-1 in cystogenesis and tubulogenesis using the canine MDCK cell line...
Long-lasting arrest of murine polycystic kidney disease with CDK inhibitor roscovitineNikolay O Bukanov
Cell Biology, Genzyme Corporation, 5 Mountain Road, Framingham, Massachusetts 01701-9322, USA
Nature 444:949-52. 2006..Our results indicate that inhibition of CDK is a new and effective approach to the treatment of PKD...
Impaired formation of desmosomal junctions in ADPKD epitheliaRyan J Russo
Genzyme Corporation, 5 Mountain Road, Framingham, MA 01701-9322, USA
Histochem Cell Biol 124:487-97. 2005..We propose that abnormal expression of PC-1 causes disregulation of cellular adhesion complexes leading to increased proliferation, loss of polarity and, ultimately, cystogenesis...
New insights into ADPKD molecular pathways using combination of SAGE and microarray technologiesHerve Husson
Functional Genomics, Genzyme Corporation, Framingham, MA 01701 9322, USA
Genomics 84:497-510. 2004..01. This study may provide valuable insights into the pathophysiology of ADPKD and suggest potential therapeutic targets...
Mechanical stimuli induce cleavage and nuclear translocation of the polycystin-1 C terminusVeronique Chauvet
Department of Cellular and Molecular Physiology, Yale University School of Medicine, New Haven, Connecticut 06510, USA
J Clin Invest 114:1433-43. 2004..Polycystin-2, the product of the second gene mutated in ADPKD, modulates the signaling properties of the polycystin-1 CTT. These data reveal a novel pathway by which polycystin-1 transmits messages directly to the nucleus...
Molecular pathogenesis of ADPKD and development of targeted therapeutic optionsOxana Ibraghimov-Beskrovnaya
Nephrol Dial Transplant 22:3367-70. 2007
Functional analysis of PKD1 transgenic lines reveals a direct role for polycystin-1 in mediating cell-cell adhesionAndrew J Streets
Sheffield Kidney Institute, Division of Clinical Sciences North, University of Sheffield, Clinical Sciences Centre, Northern General Hospital, Herries Road, Sheffield S5 7AU, UK
J Am Soc Nephrol 14:1804-15. 2003..Disruption of cell-cell adhesion during tubular morphogenesis may be an early initiating event for cyst formation in ADPKD...
Cation channel regulation by COOH-terminal cytoplasmic tail of polycystin-1: mutational and functional analysisDavid H Vandorpe
Molecular Medicine, Beth Israel Deaconess Medical Center, Boston 02215, USA
Physiol Genomics 8:87-98. 2002..The divalent cation permeability and pharmacological profile of the current has been extended. Inducible expression of the PKD1 fusion in EcR-293 cells was also associated with activation of cation channels and increased Ca2+ entry...
