Gene and Protein Information
Species	TM	AA	Chromosomal Location	Gene Symbol	Gene Name	Reference
Human	-	256	19p13.3	PRTN3	proteinase 3
Mouse	-	254	10 39.72 cM	Prtn3	proteinase 3
Rat	-	254	7q11	Prtn3	proteinase 3

Previous and Unofficial Names

AGP7 | ACPA | Wegener granulomatosis autoantigen | C-ANCA | MBT | myeloblastin | PR3

Database Links
Specialist databases
MEROPS	S01.134 (Hs)
Other databases
Alphafold	P24158 (Hs), Q61096 (Mm)
BRENDA	3.4.21.76
ChEMBL Target	CHEMBL3900 (Hs), CHEMBL5384 (Mm)
Ensembl Gene	ENSG00000196415 (Hs), ENSMUSG00000057729 (Mm), ENSRNOG00000029814 (Rn)
Entrez Gene	5657 (Hs), 19152 (Mm), 314615 (Rn)
Human Protein Atlas	ENSG00000196415 (Hs)
KEGG Enzyme	3.4.21.76
KEGG Gene	hsa:5657 (Hs), mmu:19152 (Mm), rno:314615 (Rn)
OMIM	177020 (Hs)
Orphanet	ORPHA118087 (Hs)
Pharos	P24158 (Hs)
RefSeq Nucleotide	NM_002777 (Hs), NM_011178 (Mm), NM_001024264 (Rn)
RefSeq Protein	NP_002768 (Hs), NP_035308 (Mm), NP_001019435 (Rn)
UniProtKB	P24158 (Hs), Q61096 (Mm)
Wikipedia	PRTN3 (Hs)

Selected 3D Structures
	Description:  X-ray crystal structure of human proteinase 3 (PR3). PDB Id:  1FUJ Resolution:  2.2Å Species:  Human References:  2

Enzyme Reaction
EC Number: 3.4.21.76

Download all structure-activity data for this target as a CSV file

Inhibitors
Key to terms and symbols	View all chemical structures	Click column headers to sort
Ligand Sp. Action Value Parameter Reference compound 11 [PMID: 29442501] Hs Inhibition 8.3 pKi 3 ⤷ pKi 8.3 (Ki 5.4x10-9 M) [3] Description: Inhibition of human PR3 in vitro. azapro-3 Hs Inhibition 5.8 pKi 1 ⤷ pKi 5.8 (Ki 1.5x10-6 M) [1] compound 4g [PMID: 22595175] Hs Inhibition 5.8 pIC50 5 ⤷ pIC50 5.8 (IC50 1.4x10-6 M) [5]

Immunopharmacology Comments

Proteinase 3 (PR3), called myeloblastin when it was first identified, is an abundant serine protease found principally in neutrophil granules (but is also found on the surface of quiescent human neutrophils from peripheral blood). It is stored in the primary granules of circulating neutrophils alongside other cathepsin C-activated neutrophil serine proteases (NSPs), such as human neutrophil elastase (HNE), CatG, and NSP4. In pathological conditions it is thought that PR3 behaves to accelerate inflammation, by enhancing cytokine bioactivity, inactivating anti-inflammatory mediators and by promoting tissue injury (potentially by degrading extra-cellular matrix components like elastin, collagen, fibronectin, and laminins). In addition, imbalances between NSPs and their endogenous inhibitors can contribute towards pathological tissue damage, such as the damage associated with inflammatory lung diseases like chronic obstructive pulmonary disease (COPD), emphysema, and cystic fibrosis. PR3 inhibitors are considered to be useful clinical candidates for anti-inflammatory drug development [4].

Cell Type Associations

Immuno Cell Type:  Granulocytes Cell Ontology Term:   neutrophil (CL:0000775)

Immuno Process Associations

Immuno Process:  Inflammation

Immuno Process:  Chemotaxis & migration

Immuno Process:  Immune system development

Immuno Process:  Cytokine production & signalling

Immuno Process:  Cellular signalling

Clinically-Relevant Mutations and Pathophysiology
Disease:  Wegener granulomatosis Synonyms: Wegener's granulomatosis [Disease Ontology: DOID:12132] Disease Ontology: DOID:12132 OMIM: 608710 Orphanet: ORPHA900