Neutrophil elastase

Homo sapiensTaxon: 9606

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

Sequence Length

267

amino acids

Molecular Weight

28.5 kDa

theoretical

Experimental Structures

PDB entries

Related Diseases

recorded

Function

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)

Localization & Distribution

Tissue Specificity

Bone marrow cells. Neutrophil (PubMed:10947984)

Related Diseases

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.

Amino Acid Sequence

MTLGRRLACLFLACVLPALLLGGTALASEIVGGRRARPHAWPFMVSLQLRGGHFCGATLI
APNFVMSAAHCVANVNVRAVRVVLGAHNLSRREPTRQVFAVQRIFENGYDPVNLLNDIVI
LQLNGSATINANVQVAQLPAQGRRLGNGVQCLAMGWGLLGRNRGIASVLQELNVTVVTSL
CRRSNVCTLVRGRQAGVCFGDSGSPLVCNGLIHGIASFVRGGCASGLYPDAFAPVAQFVN

FASTA format · 267 amino acids · mass 28.5 kDa

Experimental Structures

37 PDB entries

1B0F1H1B1HNE1PPF1PPG2RG32Z7F3Q763Q774NZL4WVP5A095A0A5A0B5A0C5A8X5A8Y5A8Z5ABW6E696F5M6SMA7CBK7WHU8D4Q8D4U8D7I8D7K8G248G258G268QGX8VK59ASS9ASX9ATK9ATU