title: Fibrillin degradation by matrix metalloproteinases: identification of amino- and carboxy-terminal cleavage sites.
abstract: Fibrillin molecules form the structural framework of elastic fibrillin-rich microfibrils of the extracellular matrix. We have investigated the proteolysis of recombinant fibrillin molecules by five matrix metalloproteinases. Cleavage sites were defined at the carboxy-terminal end of the fibrillin-1 proline-rich region and the corresponding fibrillin-2 glycine-rich region (exon 10), and within exon 49 towards the carboxy-terminus of fibrillin-1. Cleavage at these sites is predicted to disrupt the structure and function of the fibrillin-rich microfibrils.
authors.affiliation: Wellcome Trust Centre for Cell-Matrix Research, School of Biological Sciences, University of Manchester, UK.
authors.forename: V J
authors.initials: VJ
authors.lastname: Hindson
authors.forename: J L
authors.initials: JL
authors.lastname: Ashworth
authors.forename: M J
authors.initials: MJ
authors.lastname: Rock
authors.forename: S
authors.initials: S
authors.lastname: Cunliffe
authors.forename: C A
authors.initials: CA
authors.lastname: Shuttleworth
authors.forename: C M
authors.initials: CM
authors.lastname: Kielty
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D001665:Binding Sites; D019556:COS Cells; D002135:Calcium-Binding Proteins / Q000737:chemistry / Q000378:metabolism; D003001:Cloning, Molecular; D018076:DNA, Complementary; D016326:Extracellular Matrix Proteins / Q000737:chemistry / Q000378:metabolism; D000071837:Fibrillins; D008666:Metalloendopeptidases / Q000378:metabolism*; D008840:Microfilament Proteins / Q000737:chemistry* / Q000235:genetics / Q000378:metabolism*; D008969:Molecular Sequence Data; D010446:Peptide Fragments / Q000737:chemistry / Q000378:metabolism; D011994:Recombinant Proteins / Q000737:chemistry / Q000378:metabolism; D014158:Transcription, Genetic; D014162:Transfection
chemical_list: D002135:Calcium-Binding Proteins; D018076:DNA, Complementary; D016326:Extracellular Matrix Proteins; D000071837:Fibrillins; D008840:Microfilament Proteins; D010446:Peptide Fragments; D011994:Recombinant Proteins; D008666:Metalloendopeptidases