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A disintegrin and metalloproteinase with thrombospondin motifs 13 (ADAMTS13)
- Target
- ADAMTS13
- Molecular classification
- Enzyme, Metalloprotease, Disintegrin
01
Overview
ADAMTS13 is a secreted metalloprotease enzyme that cleaves ultra-large multimers of von Willebrand factor (UL-VWF), regulating its activity and preventing excessive platelet aggregation and microvascular thrombosis. Deficiency or dysfunction leads to Thrombotic Thrombocytopenic Purpura (TTP). It is primarily synthesized by hepatic stellate cells and endothelial cells.
- Other names
- von Willebrand factor-cleaving protease (VWFCP)vWF-cleaving proteaseC9orf8ADAMTS-13vWF-CP
02
Mechanism of action
Cleaves ultra-large multimers of von Willebrand factor (UL-VWF) at the Tyr1605-Met1606 bond under conditions of high shear stress, reducing its size and pro-thrombotic potential.
03
Biological functions
Cleavage/regulation of UL-VWF multimersRegulation of von Willebrand factor activityPrevention of excessive platelet aggregationPrevention of microvascular thrombosis
04
Disease associations
Thrombotic Thrombocytopenic Purpura (TTP)Inflammatory injuryAtherosclerosis
05
Safety considerations
Potential for off-target effects if ADAMTS13 activity is dysregulated by therapeutic interventions.Risk of bleeding if ADAMTS13 activity is excessively increased.
06
Biomarkers
ADAMTS13 activity levels (for TTP diagnosis)ADAMTS13 autoantibodies (for acquired TTP diagnosis)
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