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MGP001768

UniProt Annotations

Entry Information
Gene Namekallikrein B, plasma (Fletcher factor) 1
Protein EntryKLKB1_HUMAN
UniProt IDP03952
SpeciesHuman
Comments
Comment typeDescription
Catalytic ActivityCleaves selectively Arg-|-Xaa and Lys-|-Xaa bonds, including Lys-|-Arg and Arg-|-Ser bonds in (human) kininogen to release bradykinin.
DiseasePrekallikrein deficiency (PKK deficiency) [MIM:612423]: This disorder is a blood coagulation defect. {ECO:0000269|PubMed:14652634, ECO:0000269|PubMed:17598838}. Note=The disease is caused by mutations affecting the gene represented in this entry.
Enzyme RegulationInhibited by SERPINA5. {ECO:0000269|PubMed:2844223}.
FunctionThe 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.
SimilarityBelongs to the peptidase S1 family. Plasma kallikrein subfamily. {ECO:0000255|PROSITE-ProRule:PRU00274}.
SimilarityContains 1 peptidase S1 domain. {ECO:0000255|PROSITE- ProRule:PRU00274}.
SimilarityContains 4 apple domains. {ECO:0000255|PROSITE- ProRule:PRU00315}.
Subcellular LocationSecreted.
SubunitForms 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.
Web ResourceName=SeattleSNPs; URL="http://pga.gs.washington.edu/data/klkb1/";
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