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Items: 1 to 20 of 268

1.

Fibrillin-rich microfibrils: Structural determinants of morphogenetic and homeostatic events.

Ramirez F, Dietz HC.

J Cell Physiol. 2007 Nov;213(2):326-30. Review.

PMID:
17708531
2.

Microfibrils: a cornerstone of extracellular matrix and a key to understand Marfan syndrome.

Bonetti MI.

Ital J Anat Embryol. 2009 Oct-Dec;114(4):201-24. Review.

PMID:
20578676
3.

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 1;473(7):827-38. doi: 10.1042/BJ20151108. Review.

PMID:
27026396
4.

The fibrillins.

Ramirez F, Pereira L.

Int J Biochem Cell Biol. 1999 Feb;31(2):255-9. Review.

PMID:
10216958
5.

Fibrillin: from microfibril assembly to biomechanical function.

Kielty CM, Baldock C, Lee D, Rock MJ, Ashworth JL, Shuttleworth CA.

Philos Trans R Soc Lond B Biol Sci. 2002 Feb 28;357(1418):207-17. Review.

6.

Extracellular microfibrils in development and disease.

Ramirez F, Sakai LY, Rifkin DB, Dietz HC.

Cell Mol Life Sci. 2007 Sep;64(18):2437-46.

PMID:
17585369
7.

The molecular pathogenesis of the Marfan syndrome.

Robinson PN, Booms P.

Cell Mol Life Sci. 2001 Oct;58(11):1698-707. Review.

PMID:
11706995
8.

Biogenesis and function of fibrillin assemblies.

Ramirez F, Sakai LY.

Cell Tissue Res. 2010 Jan;339(1):71-82. doi: 10.1007/s00441-009-0822-x. Review.

9.

Fibrillin-rich microfibrils: elastic biopolymers of the extracellular matrix.

Kielty CM, Wess TJ, Haston L, Ashworth JL, Sherratt MJ, Shuttleworth CA.

J Muscle Res Cell Motil. 2002;23(5-6):581-96. Review.

PMID:
12785107
10.

Fibrillin-rich microfibrils - structural and instructive determinants of mammalian development and physiology.

Ramirez F, Carta L, Lee-Arteaga S, Liu C, Nistala H, Smaldone S.

Connect Tissue Res. 2008;49(1):1-6. doi: 10.1080/03008200701820708. Review.

PMID:
18293172
11.

Fibrillin microfibrils.

Kielty CM, Sherratt MJ, Marson A, Baldock C.

Adv Protein Chem. 2005;70:405-36. Review.

PMID:
15837522
12.

Marfan syndrome: new clues to genotype-phenotype correlations.

Ramirez F, Gayraud B, Pereira L.

Ann Med. 1999 Jun;31(3):202-7. Review.

PMID:
10442675
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 R, Suda N, Ganjargal G, Hada Y, Noguchi T, Teranaka T, Sekiguchi K, Yoneda T, Tsuji T.

J Biol Chem. 2011 Nov 4;286(44):38602-13. doi: 10.1074/jbc.M111.243451.

14.

Recent progress towards a molecular understanding of Marfan syndrome.

Dietz HC, Loeys B, Carta L, Ramirez F.

Am J Med Genet C Semin Med Genet. 2005 Nov 15;139C(1):4-9. Review.

PMID:
16273535
15.

Mutations in the human gene for fibrillin-1 (FBN1) in the Marfan syndrome and related disorders.

Dietz HC, Pyeritz RE.

Hum Mol Genet. 1995;4 Spec No:1799-809. Review.

PMID:
8541880
16.
17.

Fibrillin-1, a calcium binding protein of extracellular matrix.

Handford PA.

Biochim Biophys Acta. 2000 Dec 20;1498(2-3):84-90. Review.

18.

Structure and function of the mammalian fibrillin gene family: implications for human connective tissue diseases.

Davis MR, Summers KM.

Mol Genet Metab. 2012 Dec;107(4):635-47. doi: 10.1016/j.ymgme.2012.07.023. Review.

PMID:
22921888
19.

The molecular genetics of Marfan syndrome and related microfibrillopathies.

Robinson PN, Godfrey M.

J Med Genet. 2000 Jan;37(1):9-25. Review.

20.

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;8(1):e1002425. doi: 10.1371/journal.pgen.1002425.

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