Reactome | factor XII -> factor XIIa

factor XII -> factor XIIa

Stable Identifier

R-HSA-158313

Type

Reaction [transition]

Species

Homo sapiens

Compartment

extracellular region, plasma membrane

ReviewStatus

5/5

Locations in the PathwayBrowser

Expand all

General

SBML | | PDF

SVG | | PPTX | SBGN

Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

Cleavage of a single peptide bond converts factor XII to activated factor XII (factor XIIa) (Fujikawa and McMullen 1983; McMullen and Fujikawa 1985). Identification of the catalytic activity or activities responsible for this cleavage has not been straightforward. Studies in vitro have demonstrated the autoactivation of factor XII as well as activation by kallikrein. Both reactions require the presence of negatively charged surfaces and are accelerated in the presence of kininogen (high molecular weight kininogen, HK) (Griffin and Cochrane 1976; Meier et al. 1977; Silverberg et al. 1980). Recent work suggests that factor XII activation in vivo may occur primarily on endothelial cell surfaces and that, as in vitro, association with kininogen may accelerate the reaction (Mahdi et al. 2002; Schmaier 2004), although alternative pathways and alternative mechanisms for associating factor XII with the cell surface have not been excluded (Joseph et al. 2001).

Literature References

PubMed ID	Title	Journal	Year
11204562	Factor XII-dependent contact activation on endothelial cells and binding proteins gC1qR and cytokeratin 1 Ghebrehiwet, B, Joseph, K, Kaplan, AP, Shibayama, Y	Thromb Haemost	2001
14691562	The physiologic basis of assembly and activation of the plasma kallikrein/kinin system Schmaier, AH	Thromb Haemost	2004
874082	Activation and function of human Hageman factor. The role of high molecular weight kininogen and prekallikrein. Meier, HL, Pierce, JV, Kaplan, AP, Colman, RW	J Clin Invest	1977
1066663	Mechanisms for the involvement of high molecular weight kininogen in surface-dependent reactions of Hageman factor Cochrane, CG, Griffin, JH	Proc Natl Acad Sci U S A	1976
6604055	Amino acid sequence of human beta-factor XIIa Fujikawa, K, McMullen, BA	J Biol Chem	1983
7391081	Autoactivation of human Hageman factor. Demonstration utilizing a synthetic substrate. Silverberg, M, Dunn, JT, Kaplan, AP, Garen, L	J Biol Chem	1980
11986212	Factor XII interacts with the multiprotein assembly of urokinase plasminogen activator receptor, gC1qR, and cytokeratin 1 on endothelial cell membranes Schmaier, AH, Shariat-Madar, Z, Figueroa, CD, Mahdi, F	Blood	2002
3886654	Amino acid sequence of the heavy chain of human alpha-factor XIIa (activated Hageman factor) Fujikawa, K, McMullen, BA	J Biol Chem	1985