Table 3. FXI substrate cleavage... | American Society of Hematology

Table 3.

FXI substrate cleavage sequences, separated by physiological process

Pathway	FXIa substrate	Cleavage sequence	Reference
Coagulation	FIX	KLTR¹⁴⁵-AET DFTR¹⁸⁰-VVG	75,76
	FVIII	IQIR³⁷²-SVA IEPR⁷⁴⁰-SFS EITR¹⁶⁵²-TTL LRNR¹⁷⁴⁰-AQS	77
	FV	KKYR³⁴⁸-SQH GIQR⁵¹⁰-AAD SSWR¹⁷⁶⁵-LTS	25,77
	FX	NNLTR²³⁴-IVG	25,29
	TFPI	RIIK⁸⁶-TTL GICR¹⁰⁷-GYI GLCR¹⁹⁹-ANE	78
Platelet activation	ADAMTS13	Unknown	79
KKS	FXII	SMTR³⁷²-VVG	80
KKS	HK	SLMK³⁸⁰-RPP SPFR³⁸⁹-SSR	81
Inflammation	CFH	ENMR³⁴¹-RPY	82
	β2GPI	AFWK³³⁶-TDA	83
	Properdin	Unknown	84
	Chem163S	AFSK¹⁵⁸-ALP	85
	HGF	DLHR⁴²⁴-HIF KQLR⁴⁹⁴-VVN	86

Pathway	FXIa substrate	Cleavage sequence	Reference
Coagulation	FIX	KLTR¹⁴⁵-AET DFTR¹⁸⁰-VVG	75,76
	FVIII	IQIR³⁷²-SVA IEPR⁷⁴⁰-SFS EITR¹⁶⁵²-TTL LRNR¹⁷⁴⁰-AQS	77
	FV	KKYR³⁴⁸-SQH GIQR⁵¹⁰-AAD SSWR¹⁷⁶⁵-LTS	25,77
	FX	NNLTR²³⁴-IVG	25,29
	TFPI	RIIK⁸⁶-TTL GICR¹⁰⁷-GYI GLCR¹⁹⁹-ANE	78
Platelet activation	ADAMTS13	Unknown	79
KKS	FXII	SMTR³⁷²-VVG	80
KKS	HK	SLMK³⁸⁰-RPP SPFR³⁸⁹-SSR	81
Inflammation	CFH	ENMR³⁴¹-RPY	82
	β2GPI	AFWK³³⁶-TDA	83
	Properdin	Unknown	84
	Chem163S	AFSK¹⁵⁸-ALP	85
	HGF	DLHR⁴²⁴-HIF KQLR⁴⁹⁴-VVN	86

FXIa has a preference for cleaving substrates at peptide bonds between arginine or lysine and alanine, valine, or serine residues. To a lesser extent, FXIa can also cleave between arginine or lysine and threonine or arginine residues.

CFH, complement factor H; Chem163S, prochemerin; HGF, heptaocyte growth factor; KKS, kallikrein-kinin system; TFPI, tissue factor pathway inhibitor.

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