Proteinase 3

Proteinase 3, also known as PRTN3, is an enzyme that in humans is encoded by the PRTN3 gene.^[5]

PRTN3
Available structures PDB Ortholog search: PDBe RCSB List of PDB id codes 1FUJ
Identifiers
Aliases	PRTN3, ACPA, AGP7, C-ANCA, CANCA, MBN, MBT, NP-4, NP4, P29, PR-3, PR3, proteinase 3
External IDs	OMIM: 177020; MGI: 893580; HomoloGene: 120934; GeneCards: PRTN3; OMA:PRTN3 - orthologs
Gene location (Human) Chr. Chromosome 19 (human)[1] Band 19p13.3 Start 840,999 bp[1] End 848,175 bp[1]
Gene location (Mouse) Chr. Chromosome 10 (mouse)[2] Band 10 C1|10 39.72 cM Start 79,710,310 bp[2] End 79,719,008 bp[2]
RNA expression pattern Bgee Human Mouse (ortholog) Top expressed in bone marrow bone marrow cell spleen blood monocyte granulocyte right lung superior frontal gyrus right frontal lobe dorsolateral prefrontal cortex Top expressed in tibiofemoral joint granulocyte fetal liver hematopoietic progenitor cell body of femur bone marrow ankle joint blastocyst embryo human fetus embryo More reference expression data BioGPS More reference expression data
Gene ontology Molecular function enzyme binding peptidase activity serine-type peptidase activity hydrolase activity protein binding serine-type endopeptidase activity signaling receptor binding Cellular component extracellular matrix plasma membrane extracellular exosome cytosol extracellular region extracellular space azurophil granule lumen plasma membrane raft membrane membrane raft collagen-containing extracellular matrix Biological process collagen catabolic process blood coagulation negative regulation of phagocytosis mature conventional dendritic cell differentiation positive regulation of cell population proliferation antimicrobial humoral response neutrophil degranulation proteolysis membrane protein ectodomain proteolysis positive regulation of GTPase activity cell-cell junction maintenance neutrophil extravasation phagocytosis cytokine-mediated signaling pathway Sources:Amigo / QuickGO
Orthologs Species Human Mouse Entrez 5657 19152 Ensembl ENSG00000277804 ENSG00000196415 ENSMUSG00000057729 UniProt P24158 Q61096 RefSeq (mRNA) NM_002777 NM_011178 RefSeq (protein) NP_002768 NP_035308 Location (UCSC) Chr 19: 0.84 – 0.85 Mb Chr 10: 79.71 – 79.72 Mb PubMed search [3] [4]
Wikidata
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Function

PRTN3 is a serine protease enzyme expressed mainly in neutrophil granulocytes. Its exact role in the function of the neutrophil is unknown, but, in human neutrophils, proteinase 3 contributes to the proteolytic generation of antimicrobial peptides. It is also the target of anti-neutrophil cytoplasmic antibodies (ANCAs) of the c-ANCA (cytoplasmic subtype) class, a type of antibody frequently found in the disease granulomatosis with polyangiitis.^[6]