C1-inhibitor polymers activate the FXII-dependent kallikrein-kinin system: Implication for a role in hereditary angioedema.

Daniel Elenius Madsen,Johannes Jakobsen Sidelmann,Daniel Biltoft,Jørgen Gram,Soren Hansen

Biochimica et Biophysica Acta (BBA) - General Subjects（2015）

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摘要

•C1-inh polymers mediate FXII dependent activation of the kallikrein–kinin system.•Upon polymerization the C1-inh loses its inhibitory capacity.•HAE patients with C1-inh polymers show increased (pre)kallikrein consumption.

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关键词

BSA,C1-inh,C1PIV,CPDA,CPK,FXII,FXIIa,dBSA,HAE,HMWK,HRP,KKS,MAb,O/N,OD,OPD,PAGE,PBS,PBS-TW,PBS-TW skim,PK,PVDF,RCL,RT,SDS,Serpin,WB,FXIIa

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