4jk5
Human urokinase-type Plasminogen Activator (uPA) in complex with a bicyclic peptide inhibitor (UK18-D-Ser)Human urokinase-type Plasminogen Activator (uPA) in complex with a bicyclic peptide inhibitor (UK18-D-Ser)
Template:ABSTRACT PUBMED 23828687
DiseaseDisease
[UROK_HUMAN] Defects in PLAU are the cause of Quebec platelet disorder (QPD) [MIM:601709]. 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.[1]
FunctionFunction
[UROK_HUMAN] Specifically cleaves the zymogen plasminogen to form the active enzyme plasmin.
About this StructureAbout this Structure
4jk5 is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
ReferenceReference
- ↑ Chen S, Gfeller D, Buth SA, Michielin O, Leiman PG, Heinis C. Improving Binding Affinity and Stability of Peptide Ligands by Substituting Glycines with D-Amino Acids. Chembiochem. 2013 Jul 4. doi: 10.1002/cbic.201300228. PMID:23828687 doi:10.1002/cbic.201300228
- ↑ Paterson AD, Rommens JM, Bharaj B, Blavignac J, Wong I, Diamandis M, Waye JS, Rivard GE, Hayward CP. Persons with Quebec platelet disorder have a tandem duplication of PLAU, the urokinase plasminogen activator gene. Blood. 2010 Feb 11;115(6):1264-6. doi: 10.1182/blood-2009-07-233965. Epub 2009, Dec 9. PMID:20007542 doi:10.1182/blood-2009-07-233965