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Metalloproteinases and their inhibitors in the pathophysiology of heritable connective tissue disorders: current evidence

Authors Delhon L, Cormier-Daire V, Le Goff C

Received 22 September 2015

Accepted for publication 3 December 2015

Published 4 February 2016 Volume 2016:3 Pages 1—9

DOI https://doi.org/10.2147/MNM.S63634

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Sreejesh Shanker

Peer reviewer comments 3

Editor who approved publication: Dr Yoshifumi Itoh


Laure Delhon, Valerie Cormier-Daire, Carine Le Goff

INSERM UMR 1163, Laboratory of Molecular and Physiopathological Bases of Osteochondrodysplasias, Imagine Institute, Paris Descartes University, Paris, France

Abstract: Extracellular matrix is composed of macromolecules constituting a complex network that allows the regulation of major cellular functions and maintenance of normal tissue homeostasis. The turnover of extracellular matrix is mediated by matrix-degrading enzymes such as metalloproteinases during normal and pathological conditions. Matrix metalloproteinases and a disintegrin and metalloproteinase with thrombospondin repeats (ADAMTS) are metalloproteinases that have been identified in distinct genetic disorders. The aim of our review was to emphasize the role of the metalloproteinases based on heritable connective tissue disorder phenotypes identified in relation with ADAMTS, ADAMTS-like, or metalloproteinases mutations.

Keywords: MMP, ADAMTS, connective tissue, collagen, disorder

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