ADAMTS1 as a metalloproteinase has multiple extracellular matrix proteoglycan substrates such as aggrecan, which it cleaves at multiple sites, versican V1, nidogen-1 and 2 and tissue factor pathway inhibitor-2 (TFPI-2), which may have implications in ECM remodelling in pathological conditions such as atherosclerosis or tumor progression [22,23,24,25]. Here, TFPI2 is linked to atherosclerosis.