background:
Alpha 2 Antiplasmin is a member of the serpin family of serine protease inhibitors. The protein is a major inhibitor of plasmin, which degrades fibrin and various other proteins. Consequently, the proper function of this gene has a major role in regulating the blood clotting pathway. Mutations in this gene result in alpha-2-plasmin inhibitor deficiency, which is characterized by severe hemorrhagic diathesis. Multiple transcript variants encoding different isoforms have been found for this gene.
Function:
Serine protease inhibitor. The major targets of this inhibitor are plasmin and trypsin, but it also inactivates matriptase-3/TMPRSS7 and chymotrypsin.
Subunit:
Forms protease inhibiting heterodimer with TMPRSS7.
Subcellular Location:
Secreted.
Tissue Specificity:
Expressed by the liver and secreted in plasma.
DISEASE:
Alpha-2-plasmin inhibitor deficiency (APLID) [MIM:262850]: An autosomal recessive disorder resulting in severe hemorrhagic diathesis. Note=The disease is caused by mutations affecting the gene represented in this entry.
Similarity:
Belongs to the serpin family.
Database links:
UniProtKB/Swiss-Prot: P08697.3
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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