Essential role of a carboxyl-terminal α-helix motif in the secretion of coagulation factor XI.
Yuri HayakawaShogo TamuraNobuaki SuzukiKoya OdairaMahiru TokoroFumika KawashimaFumihiko HayakawaAkira TakagiAkira KatsumiAtsuo SuzukiShuichi OkamotoTakeshi KanematsuTadashi MatsushitaTetsuhito KojimaPublished in: Journal of thrombosis and haemostasis : JTH (2021)
FXI E597Rfs*65 results in the pathogenesis of a severe secretory defect resulting from aberrant ER-to-Golgi trafficking caused by the lack of a C-terminal α-helix motif. This study demonstrates the impact of the C-terminal structure, especially the α-helix motif, on FXI secretion.