Antithrombin-III

抗凝血酶-III

Homo sapiensTaxon: 9606

3D-structureBlood coagulationDirect protein sequencingDisease variantDisulfide bondGlycoproteinHemostasisHeparin-bindingPhosphoproteinProtease inhibitorProteomics identificationReference proteome+4

Longueur de Séquence

464

acides aminés

Poids Moléculaire

52.6 kDa

théorique

Structures Expérimentales

entrées PDB

Maladies Associées

enregistrées

Fonction

Most important serine protease inhibitor in plasma that regulates the blood coagulation cascade (PubMed:15140129, PubMed:15853774). AT-III inhibits thrombin, matriptase-3/TMPRSS7, as well as factors IXa, Xa and XIa (PubMed:15140129). Its inhibitory activity is greatly enhanced in the presence of heparin

血浆中最重要的丝氨酸蛋白酶抑制剂，可调节凝血级联 (PubMed:15140129、PubMed:15853774)。 AT-III 抑制凝血酶、matriptase-3/TMPRSS7 以及因子 IXa、Xa 和 XIa (PubMed:15140129)。在肝素存在下其抑制活性大大增强

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Localisation & Distribution

Spécificité Tissulaire

Found in plasma

Maladies Associées

Antithrombin III deficiency

An important risk factor for hereditary thrombophilia, a hemostatic disorder characterized by a tendency to recurrent thrombosis. Antithrombin-III deficiency 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.

Séquence d'Acides Aminés

MYSNVIGTVTSGKRKVYLLSLLLIGFWDCVTCHGSPVDICTAKPRDIPMNPMCIYRSPEK
KATEDEGSEQKIPEATNRRVWELSKANSRFATTFYQHLADSKNDNDNIFLSPLSISTAFA
MTKLGACNDTLQQLMEVFKFDTISEKTSDQIHFFFAKLNCRLYRKANKSSKLVSANRLFG
DKSLTFNETYQDISELVYGAKLQPLDFKENAEQSRAAINKWVSNKTEGRITDVIPSEAIN

Format FASTA · 464 acides aminés · masse 52.6 kDa

Structures Expérimentales

27 entrées PDB

1ANT1ATH1AZX1BR81DZG1DZH1E031E041E051JVQ1LK61NQ91OYH1R1L1SR51T1F1TB62ANT2B4X2B5T2BEH2GD42HIJ2ZNH3EVJ3KCG4EB1

Modifications Post-Traductionnelles

•Phosphorylated by FAM20C in the extracellular medium