Antithrombin-III

抗凝血酶-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

PDB 条目

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

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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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