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Items: 40


Dysregulated Expression of microRNA-21 and Disease-Related Genes in Human Patients and in a Mouse Model of Alport Syndrome.

Guo J, Song W, Boulanger J, Xu EY, Wang F, Zhang Y, He Q, Wang S, Yang L, Pryce C, Phillips L, MacKenna D, Leberer E, Ibraghimov-Beskrovnaya O, Ding J, Liu S.

Hum Gene Ther. 2019 Jul;30(7):865-881. doi: 10.1089/hum.2018.205. Epub 2019 Mar 29.


Casein kinase 1ε and 1α as novel players in polycystic kidney disease and mechanistic targets for (R)-roscovitine and (S)-CR8.

Billot K, Coquil C, Villiers B, Josselin-Foll B, Desban N, Delehouzé C, Oumata N, Le Meur Y, Boletta A, Weimbs T, Grosch M, Witzgall R, Saunier S, Fischer E, Pontoglio M, Fautrel A, Mrug M, Wallace D, Tran PV, Trudel M, Bukanov N, Ibraghimov-Beskrovnaya O, Meijer L.

Am J Physiol Renal Physiol. 2018 Jul 1;315(1):F57-F73. doi: 10.1152/ajprenal.00489.2017. Epub 2018 Mar 14.


Reduction of ciliary length through pharmacologic or genetic inhibition of CDK5 attenuates polycystic kidney disease in a model of nephronophthisis.

Husson H, Moreno S, Smith LA, Smith MM, Russo RJ, Pitstick R, Sergeev M, Ledbetter SR, Bukanov NO, Lane M, Zhang K, Billot K, Carlson G, Shah J, Meijer L, Beier DR, Ibraghimov-Beskrovnaya O.

Hum Mol Genet. 2016 Jun 1;25(11):2245-2255. Epub 2016 Apr 5.


Differences in the timing and magnitude of Pkd1 gene deletion determine the severity of polycystic kidney disease in an orthologous mouse model of ADPKD.

Rogers KA, Moreno SE, Smith LA, Husson H, Bukanov NO, Ledbetter SR, Budman Y, Lu Y, Wang B, Ibraghimov-Beskrovnaya O, Natoli TA.

Physiol Rep. 2016 Jun;4(12). pii: e12846. doi: 10.14814/phy2.12846.


CaMKII as a pathological mediator of ER stress, oxidative stress, and mitochondrial dysfunction in a murine model of nephronophthisis.

Bracken C, Beauverger P, Duclos O, Russo RJ, Rogers KA, Husson H, Natoli TA, Ledbetter SR, Janiak P, Ibraghimov-Beskrovnaya O, Bukanov NO.

Am J Physiol Renal Physiol. 2016 Jun 1;310(11):F1414-22. doi: 10.1152/ajprenal.00426.2015. Epub 2016 Apr 13.


CDK inhibitors R-roscovitine and S-CR8 effectively block renal and hepatic cystogenesis in an orthologous model of ADPKD.

Bukanov NO, Moreno SE, Natoli TA, Rogers KA, Smith LA, Ledbetter SR, Oumata N, Galons H, Meijer L, Ibraghimov-Beskrovnaya O.

Cell Cycle. 2012 Nov 1;11(21):4040-6. doi: 10.4161/cc.22375. Epub 2012 Oct 3.


Exome capture reveals ZNF423 and CEP164 mutations, linking renal ciliopathies to DNA damage response signaling.

Chaki M, Airik R, Ghosh AK, Giles RH, Chen R, Slaats GG, Wang H, Hurd TW, Zhou W, Cluckey A, Gee HY, Ramaswami G, Hong CJ, Hamilton BA, Cervenka I, Ganji RS, Bryja V, Arts HH, van Reeuwijk J, Oud MM, Letteboer SJ, Roepman R, Husson H, Ibraghimov-Beskrovnaya O, Yasunaga T, Walz G, Eley L, Sayer JA, Schermer B, Liebau MC, Benzing T, Le Corre S, Drummond I, Janssen S, Allen SJ, Natarajan S, O'Toole JF, Attanasio M, Saunier S, Antignac C, Koenekoop RK, Ren H, Lopez I, Nayir A, Stoetzel C, Dollfus H, Massoudi R, Gleeson JG, Andreoli SP, Doherty DG, Lindstrad A, Golzio C, Katsanis N, Pape L, Abboud EB, Al-Rajhi AA, Lewis RA, Omran H, Lee EY, Wang S, Sekiguchi JM, Saunders R, Johnson CA, Garner E, Vanselow K, Andersen JS, Shlomai J, Nurnberg G, Nurnberg P, Levy S, Smogorzewska A, Otto EA, Hildebrandt F.

Cell. 2012 Aug 3;150(3):533-48. doi: 10.1016/j.cell.2012.06.028.


Loss of GM3 synthase gene, but not sphingosine kinase 1, is protective against murine nephronophthisis-related polycystic kidney disease.

Natoli TA, Husson H, Rogers KA, Smith LA, Wang B, Budman Y, Bukanov NO, Ledbetter SR, Klinger KW, Leonard JP, Ibraghimov-Beskrovnaya O.

Hum Mol Genet. 2012 Aug 1;21(15):3397-407. doi: 10.1093/hmg/dds172. Epub 2012 May 4.


mTOR signaling in polycystic kidney disease.

Ibraghimov-Beskrovnaya O, Natoli TA.

Trends Mol Med. 2011 Nov;17(11):625-33. doi: 10.1016/j.molmed.2011.06.003. Epub 2011 Jul 19. Review.


Inhibition of glucosylceramide accumulation results in effective blockade of polycystic kidney disease in mouse models.

Natoli TA, Smith LA, Rogers KA, Wang B, Komarnitsky S, Budman Y, Belenky A, Bukanov NO, Dackowski WR, Husson H, Russo RJ, Shayman JA, Ledbetter SR, Leonard JP, Ibraghimov-Beskrovnaya O.

Nat Med. 2010 Jul;16(7):788-92. doi: 10.1038/nm.2171. Epub 2010 Jun 20.


Serum and urinary biomarker signatures for rapid preclinical in vivo assessment of CDK inhibition as a therapeutic approach for PKD.

Moreno S, Ibraghimov-Beskrovnaya O, Bukanov NO.

Cell Cycle. 2008 Jun 15;7(12):1856-64. Epub 2008 Jun 2.


CDK/GSK-3 inhibitors as therapeutic agents for parenchymal renal diseases.

Obligado SH, Ibraghimov-Beskrovnaya O, Zuk A, Meijer L, Nelson PJ.

Kidney Int. 2008 Mar;73(6):684-90. Epub 2007 Dec 19. Review.


Polycystic kidney diseases: from molecular discoveries to targeted therapeutic strategies.

Ibraghimov-Beskrovnaya O, Bukanov N.

Cell Mol Life Sci. 2008 Feb;65(4):605-19. Review.


Molecular pathogenesis of ADPKD and development of targeted therapeutic options.

Ibraghimov-Beskrovnaya O.

Nephrol Dial Transplant. 2007 Dec;22(12):3367-70. Epub 2007 Oct 30. Review. No abstract available.


Pkd1 and Nek8 mutations affect cell-cell adhesion and cilia in cysts formed in kidney organ cultures.

Natoli TA, Gareski TC, Dackowski WR, Smith L, Bukanov NO, Russo RJ, Husson H, Matthews D, Piepenhagen P, Ibraghimov-Beskrovnaya O.

Am J Physiol Renal Physiol. 2008 Jan;294(1):F73-83. Epub 2007 Oct 10.


Targeting dysregulated cell cycle and apoptosis for polycystic kidney disease therapy.

Ibraghimov-Beskrovnaya O.

Cell Cycle. 2007 Apr 1;6(7):776-9. Epub 2007 Apr 21. Review.


In vitro cystogenesis: the search for drugs antagonizing cyst development.

Ibraghimov-Beskrovnaya O, Bukanov NO.

Nephrol Ther. 2006 Jan;2 Suppl 2:S109-14.


Long-lasting arrest of murine polycystic kidney disease with CDK inhibitor roscovitine.

Bukanov NO, Smith LA, Klinger KW, Ledbetter SR, Ibraghimov-Beskrovnaya O.

Nature. 2006 Dec 14;444(7121):949-52. Epub 2006 Nov 22.


Development of polycystic kidney disease in juvenile cystic kidney mice: insights into pathogenesis, ciliary abnormalities, and common features with human disease.

Smith LA, Bukanov NO, Husson H, Russo RJ, Barry TC, Taylor AL, Beier DR, Ibraghimov-Beskrovnaya O.

J Am Soc Nephrol. 2006 Oct;17(10):2821-31. Epub 2006 Aug 23.


Impaired formation of desmosomal junctions in ADPKD epithelia.

Russo RJ, Husson H, Joly D, Bukanov NO, Patey N, Knebelmann B, Ibraghimov-Beskrovnaya O.

Histochem Cell Biol. 2005 Dec;124(6):487-97. Epub 2005 Sep 27.


Mechanical stimuli induce cleavage and nuclear translocation of the polycystin-1 C terminus.

Chauvet V, Tian X, Husson H, Grimm DH, Wang T, Hiesberger T, Igarashi P, Bennett AM, Ibraghimov-Beskrovnaya O, Somlo S, Caplan MJ.

J Clin Invest. 2004 Nov;114(10):1433-43. Erratum in: J Clin Invest. 2005 Mar;115(3):788. Hieseberger, Thomas [corrected to Hiesberger, Thomas].


New insights into ADPKD molecular pathways using combination of SAGE and microarray technologies.

Husson H, Manavalan P, Akmaev VR, Russo RJ, Cook B, Richards B, Barberio D, Liu D, Cao X, Landes GM, Wang CJ, Roberts BL, Klinger KW, Grubman SA, Jefferson DM, Ibraghimov-Beskrovnaya O.

Genomics. 2004 Sep;84(3):497-510.


The N-terminal extracellular domain is required for polycystin-1-dependent channel activity.

Babich V, Zeng WZ, Yeh BI, Ibraghimov-Beskrovnaya O, Cai Y, Somlo S, Huang CL.

J Biol Chem. 2004 Jun 11;279(24):25582-9. Epub 2004 Apr 1.


Functional analysis of PKD1 transgenic lines reveals a direct role for polycystin-1 in mediating cell-cell adhesion.

Streets AJ, Newby LJ, O'Hare MJ, Bukanov NO, Ibraghimov-Beskrovnaya O, Ong AC.

J Am Soc Nephrol. 2003 Jul;14(7):1804-15.


Canine PKD1 is a single-copy gene: genomic organization and comparative analysis.

Dackowski WR, Luderer HF, Manavalan P, Bukanov NO, Russo RJ, Roberts BL, Klinger KW, Ibraghimov-Beskrovnaya O.

Genomics. 2002 Jul;80(1):105-12.


Functional polycystin-1 expression is developmentally regulated during epithelial morphogenesis in vitro: downregulation and loss of membrane localization during cystogenesis.

O Bukanov N, Husson H, Dackowski WR, Lawrence BD, Clow PA, Roberts BL, Klinger KW, Ibraghimov-Beskrovnaya O.

Hum Mol Genet. 2002 Apr 15;11(8):923-36.


Cation channel regulation by COOH-terminal cytoplasmic tail of polycystin-1: mutational and functional analysis.

Vandorpe DH, Wilhelm S, Jiang L, Ibraghimov-Beskrovnaya O, Chernova MN, Stuart-Tilley AK, Alper SL.

Physiol Genomics. 2002 Feb 28;8(2):87-98.


A modified two-step phage display selection for isolation of polycystin-1 ligands.

Bukanov NO, Meek AL, Klinger KW, Landes GM, Ibraghimov-Beskrovnaya O.

Funct Integr Genomics. 2000 Nov;1(3):193-9.


Tuberin-dependent membrane localization of polycystin-1: a functional link between polycystic kidney disease and the TSC2 tumor suppressor gene.

Kleymenova E, Ibraghimov-Beskrovnaya O, Kugoh H, Everitt J, Xu H, Kiguchi K, Landes G, Harris P, Walker C.

Mol Cell. 2001 Apr;7(4):823-32.


Strong homophilic interactions of the Ig-like domains of polycystin-1, the protein product of an autosomal dominant polycystic kidney disease gene, PKD1.

Ibraghimov-Beskrovnaya O, Bukanov NO, Donohue LC, Dackowski WR, Klinger KW, Landes GM.

Hum Mol Genet. 2000 Jul 1;9(11):1641-9.


Cellular and subcellular distribution of polycystin-2, the protein product of the PKD2 gene.

Foggensteiner L, Bevan AP, Thomas R, Coleman N, Boulter C, Bradley J, Ibraghimov-Beskrovnaya O, Klinger K, Sandford R.

J Am Soc Nephrol. 2000 May;11(5):814-27.


Polycystin 1 is required for the structural integrity of blood vessels.

Kim K, Drummond I, Ibraghimov-Beskrovnaya O, Klinger K, Arnaout MA.

Proc Natl Acad Sci U S A. 2000 Feb 15;97(4):1731-6.


Polycystin: in vitro synthesis, in vivo tissue expression, and subcellular localization identifies a large membrane-associated protein.

Ibraghimov-Beskrovnaya O, Dackowski WR, Foggensteiner L, Coleman N, Thiru S, Petry LR, Burn TC, Connors TD, Van Raay T, Bradley J, Qian F, Onuchic LF, Watnick TJ, Piontek K, Hakim RM, Landes GM, Germino GG, Sandford R, Klinger KW.

Proc Natl Acad Sci U S A. 1997 Jun 10;94(12):6397-402.


Dystroglycan is essential for early embryonic development: disruption of Reichert's membrane in Dag1-null mice.

Williamson RA, Henry MD, Daniels KJ, Hrstka RF, Lee JC, Sunada Y, Ibraghimov-Beskrovnaya O, Campbell KP.

Hum Mol Genet. 1997 Jun;6(6):831-41.


Non-muscle alpha-dystroglycan is involved in epithelial development.

Durbeej M, Larsson E, Ibraghimov-Beskrovnaya O, Roberds SL, Campbell KP, Ekblom P.

J Cell Biol. 1995 Jul;130(1):79-91.


Heterogeneity of the 59-kDa dystrophin-associated protein revealed by cDNA cloning and expression.

Yang B, Ibraghimov-Beskrovnaya O, Moomaw CR, Slaughter CA, Campbell KP.

J Biol Chem. 1994 Feb 25;269(8):6040-4.


Primary structure and muscle-specific expression of the 50-kDa dystrophin-associated glycoprotein (adhalin).

Roberds SL, Anderson RD, Ibraghimov-Beskrovnaya O, Campbell KP.

J Biol Chem. 1993 Nov 15;268(32):23739-42.


Single base polymorphism in the DAG1 gene detected by DGGE and mismatch PCR.

Ibraghimov-Beskrovnaya O, Sheffield VC, Campbell KP.

Hum Mol Genet. 1993 Nov;2(11):1983. No abstract available.


Human dystroglycan: skeletal muscle cDNA, genomic structure, origin of tissue specific isoforms and chromosomal localization.

Ibraghimov-Beskrovnaya O, Milatovich A, Ozcelik T, Yang B, Koepnick K, Francke U, Campbell KP.

Hum Mol Genet. 1993 Oct;2(10):1651-7.


Primary structure of dystrophin-associated glycoproteins linking dystrophin to the extracellular matrix.

Ibraghimov-Beskrovnaya O, Ervasti JM, Leveille CJ, Slaughter CA, Sernett SW, Campbell KP.

Nature. 1992 Feb 20;355(6362):696-702.


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