This gene encodes a member of the ADAMTS (a disintegrin and metalloproteinase with thrombospondin motifs) protein family. Members of the family share several distinct protein modules, including a propeptide region, a metalloproteinase domain, a disintegrin-like domain, and a thrombospondin type 1 (TS-1) motif. Individual members of this family differ in the number of SLCterminal TS-1 motifs, and some have unique SLCterminal domains. The enzyme encoded by this gene contains eight TS-1 motifs. It may play roles in pulmonary cells during fetal development or in tumor processes through its proteolytic activity or as a molecule potentially involved in regulation of cell adhesion. [provided by RefSeq].
Function:
Metalloprotease that may play a role in the degradation of COMP. Cleaves also alpha-2 macroglobulin and aggregan. Has anti-tumorigenic properties.
Subunit:
Interacts with COMP.
Subcellular Location:
Secreted, extracellular space, extracellular matrix.
Tissue Specificity:
Expressed in skeletal muscle and fat. Detected at significant levels in fetal lung. Widely expressed in gastric carcinomas and in cancer cells of diverse origin.
Post-translational modifications:
The precursor is cleaved by a furin endopeptidase.
Subjected to an intracellular maturation process yielding a 120 kDa N-terminal fragment containing the metalloproteinase, disintegrin, one TSP type-1 and the Cys-rich domains and a 83 kDa SLCterminal fragment containing the spacer 2 and four TSP type-1 domains.
Glycosylated. Can be O-fucosylated by POFUT2 on a serine or a threonine residue found within the consensus sequence C1-X(2)-(S/T)-C2-G of the TSP type-1 repeat domains where C1 and C2 are the first and second cysteine residue of the repeat, respectively. Fucosylated repeats can then be further glycosylated by the addition of a beta-1,3-glucose residue by the glucosyltransferase, B3GALTL. Fucosylation mediates the efficient secretion of ADAMTS family members. Also can be SLCglycosylated with one or two mannose molecules on tryptophan residues within the consensus sequence W-X-X-W of the TPRs, and N-glycosylated. These other glycosylations can also facilitate secretion.
Similarity:
Contains 1 disintegrin domain.
Contains 1 peptidase M12B domain.
Contains 1 PLAC domain.
Contains 8 TSP type-1 domains.
SWISS:
P58397
Gene ID:
81792
Database links:
Entrez Gene: 81792 Human
Entrez Gene: 239337 Mouse
Omim: 606184 Human
SwissProt: P58397 Human
SwissProt: Q3UM66 Mouse
SwissProt: Q3UXR3 Mouse
SwissProt: Q811B3 Mouse
Unigene: 481865 Human
Unigene: 224825 Mouse
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