KLKB1

This gene encodes a glycoprotein that participates in the surface-dependent activation of blood coagulation, fibrinolysis, kinin generation and inflammation. The encoded preproprotein present in plasma as a non-covalent complex with high molecular weight kininogen undergoes proteolytic processing mediated by activated coagulation factor XII to generate a disulfide-linked, heterodimeric serine protease comprised of heavy and light chains. Certain mutations in this gene cause prekallikrein deficiency. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jan 2016]

Kallikrein B1

The enzyme cleaves Lys-Arg and Arg-Ser bonds. It activates, in a reciprocal reaction, factor XII after its binding to a negatively charged surface. It also releases bradykinin from HMW kininogen and may also play a role in the renin-angiotensin system by converting prorenin into renin.

Blood coagulationIEA:UniProtKB-KW
Blood coagulation, intrinsic pathwayIDA:ComplexPortal
Factor XII activationManual Assertion Based On ExperimentTAS:BHF-UCL
FibrinolysisIEA:UniProtKB-KW
Plasminogen activationManual Assertion Based On ExperimentIDA:BHF-UCL
Positive regulation of fibrinolysisManual Assertion Based On ExperimentIDA:BHF-UCL
ProteolysisManual Assertion Based On ExperimentIDA:ComplexPortal
Zymogen activationManual Assertion Based On ExperimentIDA:ComplexPortal

Secreted

Prekallikrein deficiency (PKK deficiency):
This disorder is a blood coagulation defect.