P01008
ANT3_HUMANSERPINC1
Antithrombin-III
抗凝血酶-III
Homo sapiensTaxon: 9606
3D-structureBlood coagulationDirect protein sequencingDisease variantDisulfide bondGlycoproteinHemostasisHeparin-bindingPhosphoproteinProtease inhibitorProteomics identificationReference proteome+4
配列長
464
アミノ酸
分子量
52.6 kDa
理論値
実験構造
27
PDB エントリー
関連疾患
1
記録済み
機能記述
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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局在と分布
組織特異性
Found in plasma
関連疾患
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.
アミノ酸配列
MYSNVIGTVTSGKRKVYLLSLLLIGFWDCVTCHGSPVDICTAKPRDIPMNPMCIYRSPEK KATEDEGSEQKIPEATNRRVWELSKANSRFATTFYQHLADSKNDNDNIFLSPLSISTAFA MTKLGACNDTLQQLMEVFKFDTISEKTSDQIHFFFAKLNCRLYRKANKSSKLVSANRLFG DKSLTFNETYQDISELVYGAKLQPLDFKENAEQSRAAINKWVSNKTEGRITDVIPSEAIN
FASTA 形式 · 464 アミノ酸 · 分子量 52.6 kDa
実験構造
27 PDB エントリー1ANT1ATH1AZX1BR81DZG1DZH1E031E041E051JVQ1LK61NQ91OYH1R1L1SR51T1F1TB62ANT2B4X2B5T2BEH2GD42HIJ2ZNH3EVJ3KCG4EB1
翻訳後修飾
- •Phosphorylated by FAM20C in the extracellular medium