A mechanism for hereditary angioedema with normal C1 inhibitor: an inhibitory regulatory role for the factor XII heavy chain

The plasma proteins factor XII (FXII) and prekallikrein (PK) undergo reciprocal activation to the proteases FXIIa and kallikrein by a process that is enhanced by surfaces (contact activation) and regulated by the serpin C1 inhibitor. Kallikrein cleaves high-molecular-weight kininogen (HK), releasing...

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Bibliographic Details
Published in:Blood
Main Authors: Ivanov, Ivan, Matafonov, Anton, Sun, Mao-fu, Mohammed, Bassem M., Cheng, Qiufang, Dickeson, S. Kent, Kundu, Suman, Verhamme, Ingrid M., Gruber, Andras, McCrae, Keith, Gailani, David
Format: Artigo
Language:Inglês
Published: American Society of Hematology 2019
Subjects:
Thrombosis and Hemostasis
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC6405335/
https://ncbi.nlm.nih.gov/pubmed/30591525
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1182/blood-2018-06-860270
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