title: The procollagen N-proteinases ADAMTS2, 3 and 14 in pathophysiology.
abstract: Collagen fibers are the main components of most of the extracellular matrices where they provide a structural support to cells, tissues and organs. Fibril-forming procollagens are synthetized as individual chains that associate to form homo- or hetero-trimers. They are characterized by the presence of a central triple helical domain flanked by amino and carboxy propeptides. Although there are some exceptions, these two propeptides have to be proteolytically removed to allow the almost spontaneous assembly of the trimers into collagen fibrils and fibers. While the carboxy-propeptide is mainly cleaved by proteinases from the tolloid family, the amino-propeptide is usually processed by procollagen N-proteinases: ADAMTS2, 3 and 14. This review summarizes the current knowledge concerning this subfamily of ADAMTS enzymes and discusses their potential involvement in physiopathological processes that are not directly linked to fibrillar procollagen processing.
authors: Laboratory of Connective Tissues Biology, GIGA-R, University of Liège, B-4000 Sart Tilman, Belgium.
authors: Mourad
authors: M
authors: Bekhouche
authors: Laboratory of Connective Tissues Biology, GIGA-R, University of Liège, B-4000 Sart Tilman, Belgium. Electronic address: acolige@ulg.ac.be.
authors: Alain
authors: A
authors: Colige
mesh_terms: D051722:ADAM Proteins / Q000172:deficiency / Q000235:genetics / Q000378:metabolism*; D000071096:ADAMTS Proteins; D000071121:ADAMTS4 Protein; D020533:Angiogenesis Inhibitors / Q000378:metabolism; D000818:Animals; D001808:Blood Vessels / Q000502:physiology; D004194:Disease / Q000235:genetics; D004535:Ehlers-Danlos Syndrome / Q000235:genetics; D024022:Fibrillar Collagens / Q000737:chemistry / Q000378:metabolism*; D006801:Humans; D042583:Lymphangiogenesis; D011348:Procollagen N-Endopeptidase / Q000172:deficiency / Q000235:genetics / Q000378:metabolism*; D013379:Substrate Specificity
chemical_list: D020533:Angiogenesis Inhibitors; D024022:Fibrillar Collagens; D051722:ADAM Proteins; D000071096:ADAMTS Proteins; C447060:ADAMTS14 protein, human; C434951:ADAMTS3 protein, human; C000609307:ADAMTS2 protein, human; D011348:Procollagen N-Endopeptidase; D000071121:ADAMTS4 Protein
keywords: ADAMTS; Fibrillar collagens; Procollagen N-proteinases