Jueju.Wang
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Neutrophil elastase structure
P08246
ELNE_HUMAN
ELANE

Neutrophil elastase

Homo sapiensTaxon: 9606

3D-structureCytoplasmic vesicleDirect protein sequencingDisease variantDisulfide bondGlycoproteinHydrolaseProteaseProteomics identificationReference proteomeSerine proteaseSignal+1
配列長

267

アミノ酸

分子量

28.5 kDa

理論値

実験構造

37

PDB エントリー

関連疾患

2

記録済み

機能記述

Serine protease that modifies the functions of natural killer cells, monocytes and granulocytes. Inhibits C5a-dependent neutrophil enzyme release and chemotaxis (PubMed:15140022). Promotes cleavage of GSDMB, thereby inhibiting pyroptosis (PubMed:36899106). Promotes blood coagulation (PubMed:20676107). Through the activation of the platelet fibrinogen receptor integrin alpha-IIb/beta-3, potentiates platelet aggregation induced by a threshold concentration of cathepsin G (CTSG) (PubMed:25211214, PubMed:9111081). Cleaves and thus inactivates tissue factor pathway inhibitor (TFPI) (PubMed:20676107, PubMed:25211214). Capable of killing E.coli but not S.aureus in vitro; digests outer membrane protein A (ompA) in E.coli and K.pneumoniae (PubMed:10947984)

局在と分布

組織特異性

Bone marrow cells. Neutrophil (PubMed:10947984)

関連疾患

Cyclic haematopoiesis

Autosomal dominant disease in which blood-cell production from the bone marrow oscillates with 21-day periodicity. Circulating neutrophils vary between almost normal numbers and zero. During intervals of neutropenia, affected individuals are at risk for opportunistic infection. Monocytes, platelets, lymphocytes and reticulocytes also cycle with the same frequency.

Neutropenia, severe congenital 1, autosomal dominant

A disorder of hematopoiesis characterized by maturation arrest of granulopoiesis at the level of promyelocytes with peripheral blood absolute neutrophil counts below 0.5 x 10(9)/l and early onset of severe bacterial infections.

アミノ酸配列

MTLGRRLACLFLACVLPALLLGGTALASEIVGGRRARPHAWPFMVSLQLRGGHFCGATLI
APNFVMSAAHCVANVNVRAVRVVLGAHNLSRREPTRQVFAVQRIFENGYDPVNLLNDIVI
LQLNGSATINANVQVAQLPAQGRRLGNGVQCLAMGWGLLGRNRGIASVLQELNVTVVTSL
CRRSNVCTLVRGRQAGVCFGDSGSPLVCNGLIHGIASFVRGGCASGLYPDAFAPVAQFVN

FASTA 形式 · 267 アミノ酸 · 分子量 28.5 kDa

実験構造

37 PDB エントリー
1B0F1H1B1HNE1PPF1PPG2RG32Z7F3Q763Q774NZL4WVP5A095A0A5A0B5A0C5A8X5A8Y5A8Z5ABW6E696F5M6SMA7CBK7WHU8D4Q8D4U8D7I8D7K8G248G258G268QGX8VK59ASS9ASX9ATK9ATU