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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)
丝氨酸蛋白酶抑制剂。 其主要靶标是弹性蛋白酶，但它对纤溶酶和凝血酶也具有中等亲和力。 不可逆地抑制胰蛋白酶、胰凝乳蛋白酶和纤溶酶原激活剂。 异常形式抑制血小板中胰岛素诱导的 NO 合成，缩短凝血时间，并具有针对胰岛素和纤溶酶的蛋白水解活性 可逆性胰凝乳蛋白酶抑制剂。 它还抑制弹性蛋白酶，但不抑制胰蛋白酶。 其主要生理功能是保护下呼吸道免受人白细胞弹性蛋白酶（HLE）的蛋白水解破坏
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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