Neutrophil elastase structure
P08246
ELNE_HUMAN
ELANE

Neutrophil elastase

Homo sapiensTaxon: 9606

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

267

아미노산

분자량

28.5 kDa

이론값

실험 구조

37

PDB 항목

관련 질환

2

기록됨

기능 설명

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)

국재 및 분포

조직 특이성

Bone marrow cells. Neutrophil (PubMed:10947984)

관련 질환

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.

아미노산 서열

MTLGRRLACLFLACVLPALLLGGTALASEIVGGRRARPHAWPFMVSLQLRGGHFCGATLI
APNFVMSAAHCVANVNVRAVRVVLGAHNLSRREPTRQVFAVQRIFENGYDPVNLLNDIVI
LQLNGSATINANVQVAQLPAQGRRLGNGVQCLAMGWGLLGRNRGIASVLQELNVTVVTSL
CRRSNVCTLVRGRQAGVCFGDSGSPLVCNGLIHGIASFVRGGCASGLYPDAFAPVAQFVN

FASTA 형식 · 267 아미노산 · 분자량 28.5 kDa

실험 구조

37 PDB 항목
1B0F1H1B1HNE1PPF1PPG2RG32Z7F3Q763Q774NZL4WVP5A095A0A5A0B5A0C5A8X5A8Y5A8Z5ABW6E696F5M6SMA7CBK7WHU8D4Q8D4U8D7I8D7K8G248G258G268QGX8VK59ASS9ASX9ATK9ATU