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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.
KLKB1
Protein class

Disease related genes, Enzymes, FDA approved drug targets, Human disease related genes, Plasma proteins

Predicted location

Intracellular, Secreted (different isoforms)

Single cell type specificity

Cell type enriched (Hepatocytes)

Immune cell specificity

Not detected in immune cells

Cell line specificity

Cell line enhanced (NTERA-2, SCLC-21H, U-937)

Interaction

Forms a heterodimer with SERPINA5. The zymogen is activated by factor XIIa, which cleaves the molecule into a light chain, which contains the active site, and a heavy chain, which associates with HMW kininogen. These chains are linked by one or more disulfide bonds.

Molecular function

Hydrolase, Protease, Serine protease

More Types Infomation

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For Research Use Only. Not For Clinical Use.

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