Related Product Information for SERPINC1 active protein
Antithrombin III (ATIII) is a single chain glycoprotein with a molecular weight of 58,000. It is a member of the serpin (serine protease inhibitor) super family and is considered to be the most important inhibitor in the coagulation cascade. ATIII inhibits a wide spectrum of serine proteases including thrombin, factors IXa, Xa and XIa, kallikrein, plasmin, urokinase, C1-esterase, and trypsin. The mechanism of inhibition involves the formation of a stable 1:1 complex between the active site of the protease and the scissile bond (Arg 385-Ser 386) of ATIII. The active site serine of thrombin has been shown to form a covalent intermediate with the P1 amino acid (Arg 385) of ATIII. The rate of inhibition of serine proteases by ATIII is increased to varying degrees by heparin. In the case of thrombin or factor Xa, the interaction with ATIII is enhanced 3 orders of magnitude in the presence of heparin. The interaction between ATIII and heparin involves a unique sequence of sulfated and non-sulfated monosaccharide units on heparin, and critical lysine residues on ATIII. The binding of ATIII to heparinoid structures on vascular endothelium has been demonstrated and shown to enhance the inhibition of factors IXa, Xa, and thrombin. ATIII may also function in the complement cascade. The binding of ATIII to fluid phase complement attack-complexes in sera has been demonstrated. In addition, the S protein of complement (an inhibitor of the membrane attack-complex) interferes with the ATIII/thrombin interaction. This preparation is prepared from fresh plasma using multi-step chromatographic methods.
Product Categories/Family for SERPINC1 active protein
NCBI Description
The protein encoded by this gene is a plasma protease inhibitor and a member of the serpin superfamily. This protein inhibits thrombin as well as other activated serine proteases of the coagulation system, and it regulates the blood coagulation cascade. The protein includes two functional domains: the heparin binding-domain at the N-terminus of the mature protein, and the reactive site domain at the C-terminus. The inhibitory activity is enhanced by the presence of heparin. More than 120 mutations have been identified for this gene, many of which are known to cause antithrombin-III deficiency. [provided by RefSeq, Jul 2009]
Uniprot Description
SERPINC1: Most important serine protease inhibitor in plasma that regulates the blood coagulation cascade. AT-III inhibits thrombin, matriptase-3/TMPRSS7, as well as factors IXa, Xa and XIa. Its inhibitory activity is greatly enhanced in the presence of heparin. Defects in SERPINC1 are the cause of antithrombin III deficiency (AT3D). AT3D is an important risk factor for hereditary thrombophilia, a hemostatic disorder characterized by a tendency to recurrent thrombosis. AT3D is classified into 4 types. Type I: characterized by a 50% decrease in antigenic and functional levels. Type II: has defects affecting the thrombin- binding domain. Type III: alteration of the heparin-binding domain. Plasma AT-III antigen levels are normal in type II and III. Type IV: consists of miscellaneous group of unclassifiable mutations. Belongs to the serpin family.
Protein type: Secreted, signal peptide; Secreted; Inhibitor
Chromosomal Location of Human Ortholog: 1q25.1
Cellular Component: extracellular space; plasma membrane; extracellular region
Molecular Function: serine-type endopeptidase inhibitor activity; heparin binding; protein binding; protease binding
Biological Process: negative regulation of inflammatory response; blood coagulation; response to nutrient
Disease: Antithrombin Iii Deficiency
Product Notes
The SERPINC1 serpinc1 (Catalog #
AAA634404) is an Active Protein and is intended for research purposes only. The product is available for immediate purchase.
It is sometimes possible for the material contained within the vial of
"Antithrombin III, Active Protein" to become dispersed throughout the inside of
the vial, particularly around the seal of said vial, during shipment and storage. We always
suggest centrifuging these vials
to consolidate all of the liquid away from the lid and to the bottom of the vial prior to
opening. Please be advised that
certain products may require dry ice for shipping and that, if this is the case, an
additional dry ice fee may also be
required.
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