Functional analysis of meltrin β/ADAM 19 in formation of the cardiac coronary artery

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ADAMs (a disintegrin and metalloprotease) family are known to be membrane-anchored glycoproteins that contain metalloprotease and disintegrin domains, and participate in the ectodomain shedding of various membrane-anchored proteins, growth factors, intercellular signaling molecules, and adhesion molecules. ADAMs play important roles in a variety of biological processes, including neurogenesis, fertilization, angiogenesis, cardiogenesis and cancer. Meltrin β/ADAM19 which is a member of the ADAMs deficient mice showed ventricular septal defect and immature valve formation. Meltrin β expressed in cardiac neural crest cells is required for ventricular septum and valve formations. Meltrin β-deficient neural crest cells migrated into the heart normally, however, they could not properly fuse the both ridges of the cushion tissues in the proximal outflow tract. Then this let to defect the membranous of the ventricular septum. Meltrin β-deficient mice showed anomalies of the coronary arteries which are aneurysm, surpentine, and orifice, formed coronary arteriovenous fistula that is influent into the ventricular septum and connected to the ventricle directly. In the ventricular septum the septum branch of the coronary artery seems like to form a big mass as a new another ventricle. We could find the blood flow of the septum coronary artery showed the regurgitant flow during systolic.