Altered versican cleavage in ADAMTS5 deficient mice; A novel etiology of myxomatous valve disease

• Published in: Developmental biology Vol. 357; no. 1; pp. 152 - 164
• Main Authors: Dupuis, Loren E., McCulloch, Daniel R., McGarity, Jessica D., Bahan, Alexandria, Wessels, Andy, Weber, Deidra, Diminich, A. Megan, Nelson, Courtney M., Apte, Suneel S., Kern, Christine B.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.09.2011
• Subjects: ADAM Proteins - genetics; ADAM Proteins - metabolism; ADAMTS5; ADAMTS5 Protein; adults; Animals; Bone Morphogenetic Protein 2 - genetics; Bone Morphogenetic Protein 2 - metabolism; Cell Proliferation; Endocardial cushions; Endocardium - metabolism; etiology; Extracellular matrix; Gene Expression Regulation, Developmental; heart; Heart - embryology; Heart Valve Diseases - etiology; Heart Valve Diseases - genetics; Heart Valves - embryology; Heart Valves - metabolism; heterozygosity; Mesoderm - metabolism; metalloproteinases; Mice; Mice, Transgenic; Myxomatous valves; penetrance; proteoglycans; Versican; Versicans - metabolism; PV; Myxomatous valves; ADAMTS; MV; EMT; ECM; AVC; VIC; PA; ADAMTS5; AV; αSMA; Endocardial cushions; Extracellular matrix; OFT; SLV; WT; Versican

In fetal valve maturation the mechanisms by which the relatively homogeneous proteoglycan-rich extracellular matrix (ECM) of endocardial cushions is replaced by a specialized and stratified ECM found in mature valves are not understood. Therefore, we reasoned that uncovering proteases critical for ‘...