Urokinase-type plasminogen activator
Function
Specifically cleaves the zymogen plasminogen to form the active enzyme plasmin.
Involvement in disease
Quebec platelet disorder
QPD
An autosomal dominant bleeding disorder due to a gain-of-function defect in fibrinolysis. Although affected individuals do not exhibit systemic fibrinolysis, they show delayed onset bleeding after challenge, such as surgery. The hallmark of the disorder is markedly increased PLAU levels within platelets, which causes intraplatelet plasmin generation and secondary degradation of alpha-granule proteins.
None
The disease is caused by variants affecting the gene represented in this entry.
Post-translational modifications
Phosphorylation of Ser-158 and Ser-323 abolishes proadhesive ability but does not interfere with receptor binding.
Produced as an inactive single-chain protein (pro-uPA or sc-uPA), is processed into the active disulfide-linked two-chain form of PLAU/uPA by a proteolytic event mediated, at least, by TMPRSS4.
Sequence Similarities
Belongs to the peptidase S1 family.
Tissue Specificity
Expressed in the prostate gland and prostate cancers.
Cellular localization
- Secreted
Alternative names
Urokinase-type plasminogen activator, U-plasminogen activator, uPA, PLAU