A disintegrin & metalloproteinase with thrombospondin type 1 motif 2; ADAMTS-2; ADAM-TS 2; ADAM-TS2; procollagen I/II amino propeptide-processing enzyme; procollagen I N-proteinase; PC I-NP; procollagen N-endopeptidase; pNPI (ADAMTS2, PCINP, PCPNI)

Function

• cleaves the propeptides of collagen type 1 & collagen type 2 prior to fibril assembly
• does not act on collagen type 3
• may also play a role in development independent of its role in collagen biosynthesis
• may be component of a multimeric complex
• binds specifically to collagen type 14 (putative)
• precursor is cleaved by a furin endopeptidase
• cleaves the N-propeptide of collagen-1 alpha-1 at Pro-|-Gln & of collagen-2 alpha-1 & collagen-1 alpha-2 at Ala-|-Gln

Cofactor: binds 1 Zn+2 per subunit (putative)

Structure

• spacer domain & the TSP type-1 domains are important for a tight interaction with the extracellular matrix
• contains 1 disintegrin domain
• contains 1 peptidase M12B domain
• contains 1 PLAC domain
• contains 4 TSP type-1 domains

Compartment

• secreted, extracellular space, extracellular matrix (putative)

Alternative splicing

named isoforms=2; LpNPI, SpNPI

Expression

• expressed at high level in skin, bone, tendon & aorta & at low levels in thymus & brain

Pathology

• defects in ADAMTS2 are the cause of Ehlers-Danlos syndrome type 7C

Notes

has been referred to as ADAMTS3

More general terms

• ADAMTS protease
• glycoprotein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=9509
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:9509
• OMIM: https://mirror.omim.org/entry/225410
• OMIM: https://mirror.omim.org/entry/604539
• UniProt: http://www.uniprot.org/uniprot/O95450.html