FEATURES

DESCRIPTION

Also known as BMP1_HUMAN, BMP1, PCOLC. Metalloprotease that plays key roles in regulating the formation of the extracellular matrix (ECM) via processing of various precursor proteins into mature functional enzymes or structural proteins (PubMed:33206546). Thereby participates in several developmental and physiological processes such as cartilage and bone formation, muscle growth and homeostasis, wound healing and tissue repair (PubMed:33169406, PubMed:32636307). Roles in ECM formation include cleavage of the C-terminal propeptides from procollagens such as procollagen I, II and III or the proteolytic activation of the enzyme lysyl oxidase LOX, necessary to formation of covalent cross-links in collagen and elastic fibers (PubMed:31152061, PubMed:33206546). Additional substrates include matricellular thrombospondin-1/THBS1 whose cleavage leads to cell adhesion disruption and TGF-beta activation (PubMed:32636307)., Plays an important role in bone repair by acting as a coactivator of BMP7. Interacts with POSTN, the interaction promotes deposition on the extracellular matrix.

EXTERNAL LINKS

BMP1

Mutations

Mutation(s) distribution

Mutations table