Physiological Functions of Plasminogen Activation: Effects of Gene Deficiencies in Humans and Mice

作者: Thomas H. Bugge

DOI: 10.1007/978-0-387-69057-5_10

关键词: ExtracellularTissue plasminogen activatorMesenchymal stem cellFibrinPlasminImmunologyCell biologyPlasminogen activatorIn vivoGeneChemistry

摘要: Exhaustive analysis of humans and mice with genetic deficiencies in plasminogen, plasminogen activators, plasmin inhibitor, activator inhibitors have yielded fundamental new insights into both the mechanisms activation physiological functions system. At least five different pathways for are operative vivo, these display a remarkable functional redundancy. Plasminogen as well components that govern inhibition system dispensable development. However, cleavage fibrin other extracellular substrates by is critical postnatal remodeling repair multiple epithelial mesenchymal tissues. As consequence, life without associated high morbidity mortality due to progressive multiorgan damage. Conversely, activators not only markedly accelerate tissue but also result lifelong bleeding predisposition premature dissolution.

参考文章(127)
J Dieval, G Nguyen, S Gross, J Delobel, EK Kruithof, A lifelong bleeding disorder associated with a deficiency of plasminogen activator inhibitor type 1 Blood. ,vol. 77, pp. 528- 532 ,(1991) , 10.1182/BLOOD.V77.3.528.528
K. Danø, P.A. Andreasen, J. Grøndahl-Hansen, P. Kristensen, L.S. Nielsen, L. Skriver, Plasminogen activators, tissue degradation, and cancer. Advances in Cancer Research. ,vol. 44, pp. 139- 266 ,(1985) , 10.1016/S0065-230X(08)60028-7
Keith W. Kombrinck, Adam H. Kaufman, Adam H. Kaufman, Angela F. Drew, Jay L. Degen, Heidi L. Schiman, Thomas H. Bugge, Persistent Corneal Haze after Excimer Laser Photokeratectomy in Plasminogen-Deficient Mice Investigative Ophthalmology & Visual Science. ,vol. 41, pp. 67- 72 ,(2000)
William P. Fay, Andrew C. Parker, Lorraine R. Condrey, Amy D. Shapiro, Human Plasminogen Activator Inhibitor-1 (PAI-1) Deficiency: Characterization of a Large Kindred With a Null Mutation in the PAI-1 Gene Blood. ,vol. 90, pp. 204- 208 ,(1997) , 10.1182/BLOOD.V90.1.204
LA Miles, EF Plow, KJ Donnelly, C Hougie, JH Griffin, A bleeding disorder due to deficiency of alpha 2-antiplasmin. Blood. ,vol. 59, pp. 1246- 1251 ,(1982) , 10.1182/BLOOD.V59.6.1246.BLOODJOURNAL5961246
Armed A. Hidayat, Patrick J. Riddle, Ligneous conjunctivitis. A clinicopathologic study of 17 cases. Ophthalmology. ,vol. 94, pp. 949- 959 ,(1987) , 10.1016/S0161-6420(87)33341-X
MH Lee, E Vosburgh, K Anderson, J McDonagh, Deficiency of Plasma Plasminogen Activator Inhibitor 1 Results in Hyperfibrinolytic Bleeding Blood. ,vol. 81, pp. 2357- 2362 ,(1993) , 10.1182/BLOOD.V81.9.2357.2357
Volker Schuster, Silvia Seidenspinner, Petra Zeitler, Cornelia Escher, Uwe Pleyer, Wolfgang Bernauer, E. Richard Stiehm, Sherwin Isenberg, Stefan Seregard, Thomas Olsson, Anne-Marie Mingers, Christian Schambeck, Hans Wolfgang Kreth, Compound-heterozygous mutations in the plasminogen gene predispose to the development of ligneous conjunctivitis. Blood. ,vol. 93, pp. 3457- 3466 ,(1999) , 10.1182/BLOOD.V93.10.3457.410K03_3457_3466