uPA Specifically cleave the zymogen plasminogen to form the active enzyme plasmin. Defects in PLAU are the cause of Quebec platelet disorder (QPD). QPD is 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. Belongs to the peptidase S1 family. 2 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: EC 3.4.21.73; Motility/polarity/chemotaxis; Protease; Secreted; Secreted, signal peptide
Chromosomal Location of Human Ortholog: 10q22.2
Cellular Component:  cell surface; extracellular region; extracellular space; plasma membrane; specific granule membrane; tertiary granule membrane
Molecular Function:  protein binding; serine-type endopeptidase activity
Biological Process:  blood coagulation; chemotaxis; fibrinolysis; neutrophil degranulation; plasminogen activation; positive regulation of cell migration; regulation of cell adhesion mediated by integrin; regulation of cell proliferation; regulation of signaling receptor activity; regulation of smooth muscle cell migration; regulation of smooth muscle cell-matrix adhesion; regulation of wound healing; response to hypoxia; signal transduction; smooth muscle cell migration
Disease: Alzheimer Disease; Quebec Platelet Disorder
Reference #:  P00749 (UniProtKB)
Alt. Names/Synonyms: ATF; plasminogen activator, urinary; plasminogen activator, urokinase; PLAU; u-PA; U-plasminogen activator; uPA; URK; UROK; Urokinase-type plasminogen activator; Urokinase-type plasminogen activator chain B; Urokinase-type plasminogen activator long chain A; Urokinase-type plasminogen activator short chain A
Gene Symbols: PLAU
Molecular weight: 48,507 Da
Basal Isoelectric point: 8.78  Predict pI for various phosphorylation states
Select Structure to View Below

uPA

Protein Structure Not Found.


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