| Activity |
|---|
| Catalytic type | Metallo |
| Peplist | Included in the Peplist with identifier PL00199 |
| NC-IUBMB | Subclass 3.4 (Peptidases) >> Sub-subclass 3.4.24 (Metalloendopeptidases) >> Peptidase 3.4.24.87
|
| Enzymology | BRENDA database |
| Proteolytic events | CutDB database (1 cleavage) |
| Physiology | von Willebrand factor (vWF) circulates in plasma as a group of multimers ranging from 500 kDa to 10,000 kDa. An important mechanism for depolymerization of the large multimers is limited proteolysis by a vWF-cleaving protease present in plasma. The endopeptidase is now identified as ADAMTS13. The absence or inactivation of the vWF-cleaving protease results in the accumulation of large multimers, which may cause thrombotic thrombocytopenic purpura (TPP) (Fujikawa et al., 2001; Barbot et al., 2001). Naturally-occurring human mutations in the gene cause thrombotic thrombocytopenic purpura (Levy et al., 2001), but low actity of ADAMTS13 can be also due to the presence of autoimmune IgG inhibitors (Tsai, 2002). |
| Knockout | ADAMTS13-deficient mice are viable and exhibit normal survival, but can display characteristics of thrombocytopenic purpura in response to certain challenges (Motto et al., 2005). |
|
Other databases
| WIKIPEDIA | http://en.wikipedia.org/wiki/ADAMTS13 |
