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Fig. 4. Schematic of the activation pathway for the procollagen N- and C-proteinases. This enzyme cascade is initiated by autocatalytic activation of furin or furin-like proprotein convertases in the trans-Golgi network and is responsible for the processing of procollagen to collagen by ADAMTS (N-proteinase) and tolloid (C-proteinase) family enzymes. Amino acid sequences cleaved by furin are shown between the pro- and catalytic domains of the enzymes. The signal sequence for secretory targeting is represented as a black rectangle. Abbreviations: ADAMTS, a disintegrin and metalloproteinase with thrombospondin motifs; BMP-1, bone morphogenetic protein 1; CUB domains, so-called owing to their occurrence in complement components, a sea urchin protein and BMP-1; EGF, epidermal growth factor; mTLD, mammalian tolloid; P-domain, region required for processing activity; Pro, prodomain, removal of which is required for enzyme activation; TLL-1, tolloid like 1; TSP, thrombospondin.

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