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Journal Abstract Search

205 related items for PubMed ID: 10931876

  • 1. New insights into the assembly of extracellular microfibrils from the analysis of the fibrillin 1 mutation in the tight skin mouse.
    Gayraud B, Keene DR, Sakai LY, Ramirez F.
    J Cell Biol; 2000 Aug 07; 150(3):667-80. PubMed ID: 10931876
    [Abstract] [Full Text] [Related]

  • 2. Mutant fibrillin 1 from tight skin mice increases extracellular matrix incorporation of microfibril-associated glycoprotein 2 and type I collagen.
    Lemaire R, Farina G, Kissin E, Shipley JM, Bona C, Korn JH, Lafyatis R.
    Arthritis Rheum; 2004 Mar 07; 50(3):915-26. PubMed ID: 15022335
    [Abstract] [Full Text] [Related]

  • 3. The Tight skin mouse: demonstration of mutant fibrillin-1 production and assembly into abnormal microfibrils.
    Kielty CM, Raghunath M, Siracusa LD, Sherratt MJ, Peters R, Shuttleworth CA, Jimenez SA.
    J Cell Biol; 1998 Mar 09; 140(5):1159-66. PubMed ID: 9490728
    [Abstract] [Full Text] [Related]

  • 4. Fibrillin in Marfan syndrome and tight skin mice provides new insights into transforming growth factor-beta regulation and systemic sclerosis.
    Lemaire R, Bayle J, Lafyatis R.
    Curr Opin Rheumatol; 2006 Nov 09; 18(6):582-7. PubMed ID: 17053502
    [Abstract] [Full Text] [Related]

  • 5. Marfan-like skeletal phenotype in the tight skin (Tsk) mouse.
    Barisic-Dujmovic T, Boban I, Adams DJ, Clark SH.
    Calcif Tissue Int; 2007 Oct 09; 81(4):305-15. PubMed ID: 17705049
    [Abstract] [Full Text] [Related]

  • 6. In vivo studies of mutant fibrillin-1 microfibrils.
    Charbonneau NL, Carlson EJ, Tufa S, Sengle G, Manalo EC, Carlberg VM, Ramirez F, Keene DR, Sakai LY.
    J Biol Chem; 2010 Aug 06; 285(32):24943-55. PubMed ID: 20529844
    [Abstract] [Full Text] [Related]

  • 7. Nonselective assembly of fibrillin 1 and fibrillin 2 in the rodent ocular zonule and in cultured cells: implications for Marfan syndrome.
    Beene LC, Wang LW, Hubmacher D, Keene DR, Reinhardt DP, Annis DS, Mosher DF, Mecham RP, Traboulsi EI, Apte SS.
    Invest Ophthalmol Vis Sci; 2013 Dec 23; 54(13):8337-44. PubMed ID: 24265020
    [Abstract] [Full Text] [Related]

  • 8. Marfan syndrome: new clues to genotype-phenotype correlations.
    Ramirez F, Gayraud B, Pereira L.
    Ann Med; 1999 Jun 23; 31(3):202-7. PubMed ID: 10442675
    [Abstract] [Full Text] [Related]

  • 9. A compound-heterozygous Marfan patient: two defective fibrillin alleles result in a lethal phenotype.
    Karttunen L, Raghunath M, Lönnqvist L, Peltonen L.
    Am J Hum Genet; 1994 Dec 23; 55(6):1083-91. PubMed ID: 7977366
    [Abstract] [Full Text] [Related]

  • 10. Expression of a mutant human fibrillin allele upon a normal human or murine genetic background recapitulates a Marfan cellular phenotype.
    Eldadah ZA, Brenn T, Furthmayr H, Dietz HC.
    J Clin Invest; 1995 Feb 23; 95(2):874-80. PubMed ID: 7860770
    [Abstract] [Full Text] [Related]

  • 11. Of mice and Marfan: genetic linkage analyses of the fibrillin genes, Fbn1 and Fbn2, in the mouse genome.
    Goldstein C, Liaw P, Jimenez SA, Buchberg AM, Siracusa LD.
    Mamm Genome; 1994 Nov 23; 5(11):696-700. PubMed ID: 7873879
    [Abstract] [Full Text] [Related]

  • 12. Evidence for a critical contribution of haploinsufficiency in the complex pathogenesis of Marfan syndrome.
    Judge DP, Biery NJ, Keene DR, Geubtner J, Myers L, Huso DL, Sakai LY, Dietz HC.
    J Clin Invest; 2004 Jul 23; 114(2):172-81. PubMed ID: 15254584
    [Abstract] [Full Text] [Related]

  • 13. ADAMTSL6β protein rescues fibrillin-1 microfibril disorder in a Marfan syndrome mouse model through the promotion of fibrillin-1 assembly.
    Saito M, Kurokawa M, Oda M, Oshima M, Tsutsui K, Kosaka K, Nakao K, Ogawa M, Manabe RI, Suda N, Ganjargal G, Hada Y, Noguchi T, Teranaka T, Sekiguchi K, Yoneda T, Tsuji T.
    J Biol Chem; 2011 Nov 04; 286(44):38602-38613. PubMed ID: 21880733
    [Abstract] [Full Text] [Related]

  • 14. Microenvironmental regulation by fibrillin-1.
    Sengle G, Tsutsui K, Keene DR, Tufa SF, Carlson EJ, Charbonneau NL, Ono RN, Sasaki T, Wirtz MK, Samples JR, Fessler LI, Fessler JH, Sekiguchi K, Hayflick SJ, Sakai LY.
    PLoS Genet; 2012 Jan 04; 8(1):e1002425. PubMed ID: 22242013
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  • 16. New insights into the structure, assembly and biological roles of 10-12 nm connective tissue microfibrils from fibrillin-1 studies.
    Jensen SA, Handford PA.
    Biochem J; 2016 Apr 01; 473(7):827-38. PubMed ID: 27026396
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  • 18. Characterization of mutated protein encoded by partially duplicated fibrillin-1 gene in tight skin (TSK) mice.
    Saito S, Nishimura H, Brumeanu TD, Casares S, Stan AC, Honjo T, Bona CA.
    Mol Immunol; 1999 Feb 01; 36(3):169-76. PubMed ID: 10403482
    [Abstract] [Full Text] [Related]

  • 19. Adamtsl2 deletion results in bronchial fibrillin microfibril accumulation and bronchial epithelial dysplasia--a novel mouse model providing insights into geleophysic dysplasia.
    Hubmacher D, Wang LW, Mecham RP, Reinhardt DP, Apte SS.
    Dis Model Mech; 2015 May 01; 8(5):487-99. PubMed ID: 25762570
    [Abstract] [Full Text] [Related]

  • 20. A microfibril assembly assay identifies different mechanisms of dominance underlying Marfan syndrome, stiff skin syndrome and acromelic dysplasias.
    Jensen SA, Iqbal S, Bulsiewicz A, Handford PA.
    Hum Mol Genet; 2015 Aug 01; 24(15):4454-63. PubMed ID: 25979247
    [Abstract] [Full Text] [Related]

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