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UniProt functional annotation for P05546 | ||
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| UniProt code: P05546. |
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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: | |
Thrombin inhibitor activated by the glycosaminoglycans, heparin or dermatan sulfate. In the presence of the latter, HC-II becomes the predominant thrombin inhibitor in place of antithrombin III (AT-III). Also inhibits chymotrypsin, but in a glycosaminoglycan- independent manner. {ECO:0000269|PubMed:1939083}. |
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| Function: | |
Peptides at the N-terminal of HC-II have chemotactic activity for both monocytes and neutrophils. {ECO:0000269|PubMed:1939083}. |
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| Tissue specificity: | |
Expressed predominantly in liver. Also present in plasma. |
| Domain: | |
The N-terminal acidic repeat region mediates, in part, the glycosaminoglycan-accelerated thrombin inhibition. |
| Ptm: | |
Phosphorylated by FAM20C in the extracellular medium. {ECO:0000269|PubMed:26091039}. |
| Disease: | |
Thrombophilia due to heparin cofactor 2 deficiency (THPH10) [MIM:612356]: A hemostatic disorder characterized by a tendency to recurrent thrombosis. {ECO:0000269|PubMed:10391209, ECO:0000269|PubMed:11204559, ECO:0000269|PubMed:15337701, ECO:0000269|PubMed:2647747}. Note=The disease is caused by variants affecting the gene represented in this entry. |
| Similarity: | |
Belongs to the serpin family. {ECO:0000305}. |
| Sequence caution: | |
Sequence=CAG30459.1; Type=Erroneous initiation; Note=Extended N-terminus.; Evidence={ECO:0000305}; |
Annotations taken from UniProtKB at the EBI.