ADAMTS3
ADAMTS3 shares the procollagen N-proteinase activity of ADAMTS2 and was originally characterized as procollagen II N-proteinase, but it is best known for activating the lymphangiogenic growth factor VEGF-C by proteolytic removal of its C-terminal propeptide, a modification required for signaling through VEGFR-3. A similar activation applies to VEGF-D. These functions explain the phenotype of ADAMTS3 mutations, which cause Hennekam syndrome, an autosomal recessive disorder combining congenital lymphedema, intestinal lymphangiectasia and characteristic facial features. The enzyme is expressed in embryonic cartilage and vascular tissues, and recombinant ADAMTS3 is used in lymphangiogenesis and VEGF-C biology research.
