P01008
ANT3_HUMANSERPINC1
Antithrombin-III
Homo sapiensTaxon: 9606
3D-structureBlood coagulationDirect protein sequencingDisease variantDisulfide bondGlycoproteinHemostasisHeparin-bindingPhosphoproteinProtease inhibitorProteomics identificationReference proteome+4
序列长度
464
氨基酸
分子量
52.6 kDa
理论值
实验结构
27
PDB 条目
相关疾病
1
已记录
功能描述
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
定位与分布
组织特异性
Found in plasma
相关疾病
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.
氨基酸序列
MYSNVIGTVTSGKRKVYLLSLLLIGFWDCVTCHGSPVDICTAKPRDIPMNPMCIYRSPEK KATEDEGSEQKIPEATNRRVWELSKANSRFATTFYQHLADSKNDNDNIFLSPLSISTAFA MTKLGACNDTLQQLMEVFKFDTISEKTSDQIHFFFAKLNCRLYRKANKSSKLVSANRLFG DKSLTFNETYQDISELVYGAKLQPLDFKENAEQSRAAINKWVSNKTEGRITDVIPSEAIN
FASTA 格式 · 464 个氨基酸 · 分子量 52.6 kDa
实验结构
27 PDB 条目1ANT1ATH1AZX1BR81DZG1DZH1E031E041E051JVQ1LK61NQ91OYH1R1L1SR51T1F1TB62ANT2B4X2B5T2BEH2GD42HIJ2ZNH3EVJ3KCG4EB1
翻译后修饰
- •Phosphorylated by FAM20C in the extracellular medium