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Alpha-1-antitrypsin structure
P01009
A1AT_HUMAN
SERPINA1

Alpha-1-antitrypsin

Homo sapiensTaxon: 9606

3D-structureAcute phaseAlternative splicingBlood coagulationDirect protein sequencingEndoplasmic reticulumExtracellular matrixGlycoproteinHemostasisPhosphoproteinProtease inhibitorProteomics identification+4
序列长度

418

氨基酸

分子量

46.7 kDa

理论值

实验结构

43

PDB 条目

相关疾病

1

已记录

功能描述

Inhibitor of serine proteases. Its primary target is elastase, but it also has a moderate affinity for plasmin and thrombin. Irreversibly inhibits trypsin, chymotrypsin and plasminogen activator. The aberrant form inhibits insulin-induced NO synthesis in platelets, decreases coagulation time and has proteolytic activity against insulin and plasmin Reversible chymotrypsin inhibitor. It also inhibits elastase, but not trypsin. Its major physiological function is the protection of the lower respiratory tract against proteolytic destruction by human leukocyte elastase (HLE)

定位与分布

组织特异性

Ubiquitous. Expressed in leukocytes and plasma

相关疾病

Alpha-1-antitrypsin deficiency

An autosomal recessive disorder characterized by serum levels of alpha-1-antitrypsin below the normal range, and an increased risk for developing pulmonary emphysema and, to a lesser extent, chronic liver disease. Environmental factors, particularly cigarette smoking, greatly increase the risk of emphysema at an earlier age.

氨基酸序列

MPSSVSWGILLLAGLCCLVPVSLAEDPQGDAAQKTDTSHHDQDHPTFNKITPNLAEFAFS
LYRQLAHQSNSTNIFFSPVSIATAFAMLSLGTKADTHDEILEGLNFNLTEIPEAQIHEGF
QELLRTLNQPDSQLQLTTGNGLFLSEGLKLVDKFLEDVKKLYHSEAFTVNFGDTEEAKKQ
INDYVEKGTQGKIVDLVKELDRDTVFALVNYIFFKGKWERPFEVKDTEEEDFHVDQVTTV

FASTA 格式 · 418 个氨基酸 · 分子量 46.7 kDa

实验结构

43 PDB 条目
1ATU1D5S1EZX1HP71IZ21KCT1OO81OPH1PSI1QLP1QMB2D262QUG3CWL3CWM3DRM3DRU3NDD3NDF3NE43T1P4PYW5IO15NBU5NBV6HX46I4V6I7U6IAY6ROD7AEL7API7NPK7NPL8API8P4J8P4U8PI28QZ58R138R219API9GGP

翻译后修饰

  • •N-glycosylated. Differential glycosylation produces a number of isoforms. N-linked glycan at Asn-107 is alternatively di-antennary, tri-antennary or tetra-antennary. The glycan at Asn-70 is di-antennary with trace amounts of tri-antennary. Glycan at Asn-271 is exclusively di-antennary. Structure of glycans at Asn-70 and Asn-271 is Hex5HexNAc4. The structure of the antennae is Neu5Ac(alpha1-6)Gal(beta1-4)GlcNAc attached to the core structure Man(alpha1-6)[Man(alpha1-3)]Man(beta1-4)GlcNAc(beta1-4)GlcNAc. Some antennae are fucosylated, which forms a Lewis-X determinant
  • •Proteolytic processing may yield the truncated form that ranges from Asp-30 to Lys-418
  • •(Microbial infection) Proteolytically processed by Staphylococcus aureus seryl, cysteinyl, and metallo-proteases