Antithrombin-III

抗凝血酶-III

Homo sapiensTaxon: 9606

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

서열 길이

464

아미노산

분자량

52.6 kDa

이론값

실험 구조

PDB 항목

기능 설명

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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국재 및 분포

조직 특이성

Found in plasma

관련 질환

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.

아미노산 서열

MYSNVIGTVTSGKRKVYLLSLLLIGFWDCVTCHGSPVDICTAKPRDIPMNPMCIYRSPEK
KATEDEGSEQKIPEATNRRVWELSKANSRFATTFYQHLADSKNDNDNIFLSPLSISTAFA
MTKLGACNDTLQQLMEVFKFDTISEKTSDQIHFFFAKLNCRLYRKANKSSKLVSANRLFG
DKSLTFNETYQDISELVYGAKLQPLDFKENAEQSRAAINKWVSNKTEGRITDVIPSEAIN

FASTA 형식 · 464 아미노산 · 분자량 52.6 kDa

실험 구조

27 PDB 항목

1ANT1ATH1AZX1BR81DZG1DZH1E031E041E051JVQ1LK61NQ91OYH1R1L1SR51T1F1TB62ANT2B4X2B5T2BEH2GD42HIJ2ZNH3EVJ3KCG4EB1

번역 후 수식

•Phosphorylated by FAM20C in the extracellular medium