ADAMTS6
null ADAMTS6 stands apart from the aggrecanases by its association with cardiovascular development and fibrillin-rich microfibrils. Expression studies place the gene in the embryonic heart and outflow tract, and targeted disruption of Adamts6 in mice causes mid-gestational lethality with abnormalities of the great vessels, implicating the enzyme in extracellular-matrix remodeling during cardiac morphogenesis. Reported activities include processing of fibrillin-containing microfibrillar substrates, which distinguishes ADAMTS6 from the proteoglycanase branch, although its full physiological substrate spectrum continues to be defined. Recombinant ADAMTS6 is accordingly used in studies of developmental cardiovascular biology and microfibril assembly.
