1gi8
A NOVEL SERINE PROTEASE INHIBITION MOTIF INVOLVING A MULTI-CENTERED SHORT HYDROGEN BONDING NETWORK AT THE ACTIVE SITEA NOVEL SERINE PROTEASE INHIBITION MOTIF INVOLVING A MULTI-CENTERED SHORT HYDROGEN BONDING NETWORK AT THE ACTIVE SITE
Template:ABSTRACT PUBMED 11292354
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
1gi8 is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
See AlsoSee Also
ReferenceReference
- ↑ Katz BA, Elrod K, Luong C, Rice MJ, Mackman RL, Sprengeler PA, Spencer J, Hataye J, Janc J, Link J, Litvak J, Rai R, Rice K, Sideris S, Verner E, Young W. A novel serine protease inhibition motif involving a multi-centered short hydrogen bonding network at the active site. J Mol Biol. 2001 Apr 13;307(5):1451-86. PMID:11292354 doi:http://dx.doi.org/10.1006/jmbi.2001.4516
- ↑ 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
Proteopedia Page Contributors and Editors (what is this?)Proteopedia Page Contributors and Editors (what is this?)
OCA- Homo sapiens
- U-plasminogen activator
- Elrod, K.
- Hatayte, J.
- Janc, J.
- Katz, B A.
- Link, J.
- Litvak, J.
- Luong, C.
- Mackman, R L.
- Rai, R.
- Rice, K.
- Rice, M.
- Sideris, S.
- Spencer, J.
- Sprengeler, P A.
- Verner, E.
- Young, W.
- Blood clotting
- Hydrolase
- Oxyanion hole water
- Shift of pka of his57
- Specificity
- Structure-based drug design
- Three-centered
- Thrombin
- Trypsin
- Urokinase
- Very short hydrogen bond
- Zn+2-mediated inhibition