Antithrombin III Protein | SERPINC1 protein

Antithrombin III, Human (ATIII)

Cat. Num. AAA635251

• Gene Names: SERPINC1; AT3; AT3D; ATIII; THPH7
• Purity: Affinity Purified; Purified by heparin-agarose affinity chromatography (3). Purity is determined by SDS-PAGE analysis.
• Synonyms: Antithrombin III; Human (ATIII); SERPINC1 protein

• Host: Human plasma (fresh frozen)
• Purity/Purification: Affinity Purified; Purified by heparin-agarose affinity chromatography (3). Purity is determined by SDS-PAGE analysis.
• Form/Format: Supplied as a liquid in 50% glycerol/H2O.

• Preparation and Storage: May be stored at 4 degree C for short-term only. For long-term storage, store at -20 degree C. Aliquots are stable for at least 6 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

Related Product Information for SERPINC1 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 (1,2). 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.

Product Categories/Family for SERPINC1 protein

Molecular Biology; MB-Coagulation Factors

NCBI and Uniprot Product Information

• NCBI GI #: 4502261
• NCBI GeneID: 462
• NCBI Accession #: NP_000479.1
• NCBI GenBank Nucleotide #: NM_000488.3
• UniProt Accession #: P01008; P78439; P78447; Q13815; Q5TC78; Q7KZ43; Q7KZ97; Q9UC78; B2R6P0
• Molecular Weight: 58,000
• NCBI Official Full Name: antithrombin-III
• NCBI Official Synonym Full Names: serpin peptidase inhibitor, clade C (antithrombin), member 1
• NCBI Official Symbol: SERPINC1
• NCBI Official Synonym Symbols: AT3; AT3D; ATIII; THPH7
• NCBI Protein Information: antithrombin-III; serine (or cysteine) proteinase inhibitor, clade C (antithrombin), member 1
• UniProt Protein Name: Antithrombin-III
• Protein Family: Antithrombin
• UniProt Gene Name: SERPINC1
• UniProt Synonym Gene Names: AT3; ATIII
• UniProt Entry Name: ANT3_HUMAN

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; Inhibitor; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 1q25.1

Cellular Component: extracellular space; plasma membrane; extracellular region

Molecular Function: heparin binding; serine-type endopeptidase inhibitor activity; protein binding; protease binding

Biological Process: negative regulation of inflammatory response; blood coagulation; response to nutrient

Disease: Antithrombin Iii Deficiency