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UniProt functional annotation for P03952 | ||
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| UniProt code: P03952. |
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| Organism: | |
Homo sapiens (Human). |
| Taxonomy: | |
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo. |
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| Function: | |
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. |
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| Catalytic activity: | |
Reaction=Cleaves selectively Arg-|-Xaa and Lys-|-Xaa bonds, including Lys-|-Arg and Arg-|-Ser bonds in (human) kininogen to release bradykinin.; EC=3.4.21.34; |
| Activity regulation: | |
Inhibited by SERPINA5. {ECO:0000269|PubMed:2844223}. |
| Subunit: | |
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. |
| Subcellular location: | |
Secreted. |
| Disease: | |
Prekallikrein 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 variants affecting the gene represented in this entry. |
| Similarity: | |
Belongs to the peptidase S1 family. Plasma kallikrein subfamily. {ECO:0000255|PROSITE-ProRule:PRU00274}. |
Annotations taken from UniProtKB at the EBI.