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存放说明
Store at -20°C. Please refer to protocols.
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研究领域
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功能
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.
Short peptide from AAT: 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).
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组织特异性
Ubiquitous. Expressed in leukocytes and plasma.
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疾病相关
Alpha-1-antitrypsin deficiency
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序列相似性
Belongs to the serpin family.
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结构域
The reactive center loop (RCL) extends out from the body of the protein and directs binding to the target protease. The protease cleaves the serpin at the reactive site within the RCL, establishing a covalent linkage between the carboxyl group of the serpin reactive site and the serine hydroxyl of the protease. The resulting inactive serpin-protease complex is highly stable.
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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.
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细胞定位
Secreted. Endoplasmic reticulum. The S and Z allele are not secreted effectively and accumulate intracellularly in the endoplasmic reticulum and Secreted, extracellular space, extracellular matrix.
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Information by UniProt
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别名
- AAT
- Alpha 1 antiproteinase
- Alpha 1 antitrypsin
- Alpha 1 antitrypsin null
- Alpha 1 protease inhibitor
- Alpha-1 protease inhibitor
- Alpha-1-antiproteinase
- alpha1 proteinase inhibitor
- Alpha1AT
- Dom1
- PI
- PI1
- PRO2275
- Serine (or cysteine) proteinase inhibitor clade A member 1
- Serine protease inhibitor 1-1
- Serine protease inhibitor A1a
- Serpin A1
- Serpin A1a
- Serpin peptidase inhibitor clade A member 1
- Serpina1
- Short peptide from AAT
- SPAAT
- Spi1-1
see all