PRTN3
entrez
5657
canonical
uniprot
P24158
canonical
ensembl
ENSG00000196415
resolver
ensembl
ENSG00000277804
resolver
ensp
ENSP00000234347
resolver
ensp
ENSP00000478977
resolver
enst
ENST00000234347
resolver
enst
ENST00000612112
resolver
genesymbol
PRTN3
resolver
genesymbol_syn
MBN
resolver
hgnc
HGNC:9495
resolver
uniprot
P15637
resolver
uniprot
P18078
resolver
uniprot
Q4VB08
resolver
uniprot
Q4VB09
resolver
uniprot
Q6LBM7
resolver
uniprot
Q6LBN2
resolver
uniprot
Q9UD25
resolver
uniprot
Q9UQD8
resolver
uniprot_entry
PRTN3_HUMAN
resolver
uniprot_sec
P15637
resolver
uniprot_sec
P18078
resolver
uniprot_sec
Q4VB08
resolver
uniprot_sec
Q4VB09
resolver
uniprot_sec
Q6LBM7
resolver
uniprot_sec
Q6LBN2
resolver
uniprot_sec
Q9UD25
resolver
uniprot_sec
Q9UQD8
resolver
activity_regulation
ACTIVITY REGULATION: Inhibited by phenylmethanesulfonyl fluoride (PMSF) and diisopropyl fluorophosphate (DFP). {ECO:0000269|PubMed:3198760}.
uniprot
proteins
amino_acid_sequence
MAHRPPSPALASVLLALLLSGAARAAEIVGGHEAQPHSRPYMASLQMRGNPGSHFCGGTLIHPSFVLTAAHCLRDIPQRLVNVVLGAHNVRTQEPTQQHFSVAQVFLNNYDAENKLNDVLLIQLSSPANLSASVATVQLPQQDQPVPHGTQCLAMGWGRVGAHDPPAQVLQELNVTVVTFFCRPHNICTFVPRRKAGICFGDSGGPLICDGIIQGIDSFVIWGCATRLFPDFFTRVALYVDWIRSTLRRVEAKGRP
uniprot
proteins
disease_involvement
DISEASE: Note=Is the major autoantigen in anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (Wegener's granulomatosis) (PubMed:2377228, PubMed:2679910). This complex, systemic disease is characterized by granulomatous inflammation with necrotizing lesions in the respiratory tract, glomerulonephritis, vasculitis, and anti-neutrophil cytoplasmic autoantibodies detected in patient sera (PubMed:2377228, PubMed:2679910). PRTN3 causes emphysema when administered by tracheal insufflation to hamsters (PubMed:3198760). {ECO:0000269|PubMed:3198760, ECO:0000303|PubMed:2377228, ECO:0000303|PubMed:2679910}.
uniprot
proteins
ec_number
3.4.21.76
uniprot
proteins
function
FUNCTION: Serine protease that degrades elastin, fibronectin, laminin, vitronectin, and collagen types I, III, and IV (in vitro) (PubMed:2033050, PubMed:28240246, PubMed:3198760). By cleaving and activating receptor F2RL1/PAR-2, enhances endothelial cell barrier function and thus vascular integrity during neutrophil transendothelial migration (PubMed:23202369). Plays a role in neutrophil transendothelial migration, probably when associated with CD177 (PubMed:22266279). Triggers inflammatory processes in neutrophils by interacting with ADGRG3 upstream of F2RL1/PAR2 activation (PubMed:36302784). {ECO:0000269|PubMed:2033050, ECO:0000269|PubMed:22266279, ECO:0000269|PubMed:23202369, ECO:0000269|PubMed:28240246, ECO:0000269|PubMed:3198760, ECO:0000269|PubMed:36302784}.
uniprot
proteins
mass_dalton
27807
uniprot
proteins
protein_family
Elastase subfamily
uniprot
proteins
protein_family
Peptidase S1 family
uniprot
proteins
pubmed
1400430
uniprot
proteins
pubmed
15057824
uniprot
proteins
pubmed
1518849
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
1681549
uniprot
proteins
pubmed
1688612
uniprot
proteins
pubmed
17244676
uniprot
proteins
pubmed
18462208
uniprot
proteins
pubmed
2001463
uniprot
proteins
pubmed
2033050
uniprot
proteins
pubmed
21193407
uniprot
proteins
pubmed
2121162
uniprot
proteins
pubmed
21269460
uniprot
proteins
pubmed
22266279
uniprot
proteins
pubmed
2242436
uniprot
proteins
pubmed
2258701
uniprot
proteins
pubmed
2285532
uniprot
proteins
pubmed
23202369
uniprot
proteins
pubmed
2377228
uniprot
proteins
pubmed
2404977
uniprot
proteins
pubmed
2501794
uniprot
proteins
pubmed
25944712
uniprot
proteins
pubmed
2598267
uniprot
proteins
pubmed
2679910
uniprot
proteins
pubmed
28240246
uniprot
proteins
pubmed
30692621
uniprot
proteins
pubmed
3198760
uniprot
proteins
pubmed
36302784
uniprot
proteins
pubmed
7539799
uniprot
proteins
pubmed
7897245
uniprot
proteins
pubmed
8757293
uniprot
proteins
pubmed
9924693
uniprot
proteins
sequence_length
256
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Cytoplasmic granule {ECO:0000269|PubMed:18462208, ECO:0000269|PubMed:2033050, ECO:0000269|PubMed:3198760, ECO:0000269|PubMed:7897245, ECO:0000305|PubMed:17244676}. Secreted {ECO:0000269|PubMed:18462208, ECO:0000269|PubMed:28240246}. Cell membrane {ECO:0000269|PubMed:17244676, ECO:0000269|PubMed:18462208, ECO:0000269|PubMed:22266279, ECO:0000269|PubMed:28240246}; Peripheral membrane protein {ECO:0000269|PubMed:17244676, ECO:0000269|PubMed:18462208, ECO:0000269|PubMed:22266279, ECO:0000269|PubMed:28240246}; Extracellular side {ECO:0000269|PubMed:17244676, ECO:0000269|PubMed:18462208, ECO:0000269|PubMed:22266279, ECO:0000269|PubMed:28240246}. Membrane raft {ECO:0000269|PubMed:21193407}; Peripheral membrane protein {ECO:0000269|PubMed:21193407}; Extracellular side {ECO:0000269|PubMed:21193407}. Note=Localizes predominantly to azurophil granules (primary secretory granules) in neutrophils (PubMed:18462208, PubMed:2033050, PubMed:3198760, PubMed:7897245). Secreted upon neutrophil stimulation by TNF, lipopolysaccharide (LPS), fMLP and CXCL8/IL8 or during neutrophil transmigration (PubMed:22266279, PubMed:28240246). Following secretion tethered to the cell membrane by CD177 (PubMed:18462208, PubMed:22266279). {ECO:0000269|PubMed:18462208, ECO:0000269|PubMed:2033050, ECO:0000269|PubMed:22266279, ECO:0000269|PubMed:28240246, ECO:0000269|PubMed:3198760, ECO:0000269|PubMed:7897245}.
uniprot
proteins