alpha-2-antiplasmin; alpha-2-AP; alpha-2-plasmin inhibitor; alpha-2-PI; Serpin F2 (SERPINF2, AAP, PLI)

Function: - inhibits plasmin by forming an irreversible complex with catalytic site - prevents plasmin binding to fibrin & is itself bound to fibrin by Factor XIIIa - major targets of this inhibitor are plasmin & trypsin, but it also inactivates chymotrypsin Structure: - belongs to the serpin family Compartment: secreted Expression: - expressed by the liver & secreted in plasma Pathology: - defects in SERPINF2 are the cause of alpha-2-antiplasmin deficiency

General

alpha 2 globulin serine protease inhibitor; serpin

Properties

COMPARTMENT: plasma INHIBITS: serine protease SIZE: entity length = 491 aa MW = 55 kD MOTIF: signal sequence {1-27} cysteine residue {C70} MODIFICATION: cysteine residue {C143} N-glycosylation site {N126} cysteine residue {C143} MODIFICATION: cysteine residue {C70} N-glycosylation site {N295} N-glycosylation site {N309} N-glycosylation site {N316} peptide motif {403-404} peptide motif {404-405} MISC-INFO: lifetime 2.6 DAYS CONCENTRATION 1 UM

Database Correlations

OMIM correlations MORBIDMAP 613168 UniProt P08697 Pfam PF00079 Entrez Gene 5345 Kegg hsa:5345

References

Clinical Diagnosis and Management by Laboratory Methods, 18th ed, J.B. Henry (ed), W.B. Saunders, Philadelphia, PA, 1991 pg 739