Free for academic non-profit institutions. Other users need a Commercial license

Aliases for PRTN3 Gene

Aliases for PRTN3 Gene

  • Proteinase 3 2 3 5
  • Wegener Granulomatosis Autoantigen 2 3
  • Serine Proteinase, Neutrophil 2 3
  • Neutrophil Proteinase 4 3 4
  • Leukocyte Proteinase 3 3 4
  • Wegener Autoantigen 3 4
  • C-ANCA Antigen 3 4
  • Myeloblastin 2 3
  • AGP7 3 4
  • NP-4 3 4
  • PR-3 3 4
  • MBN 3 4
  • P29 3 4
  • PR3 3 4
  • Azurophil Granule Protein 7 3
  • EC 4
  • C-ANCA 3
  • CANCA 3
  • ACPA 3
  • MBT 3
  • NP4 3

External Ids for PRTN3 Gene

Previous GeneCards Identifiers for PRTN3 Gene

  • GC19P000922
  • GC19P000910
  • GC19P000780
  • GC19P000793
  • GC19P000498
  • GC19P000794
  • GC19P000610

Summaries for PRTN3 Gene

GeneCards Summary for PRTN3 Gene

PRTN3 (Proteinase 3) is a Protein Coding gene. Diseases associated with PRTN3 include Wegener Granulomatosis and Vasculitis. Among its related pathways are Formation of Fibrin Clot (Clotting Cascade) and C-MYB transcription factor network. Gene Ontology (GO) annotations related to this gene include enzyme binding and serine-type peptidase activity. An important paralog of this gene is ELANE.

UniProtKB/Swiss-Prot for PRTN3 Gene

  • Serine protease that degrades elastin, fibronectin, laminin, vitronectin, and collagen types I, III, and IV (in vitro) (PubMed:3198760, PubMed:2033050, PubMed:28240246). By cleaving and activating receptor F2RL1/PAR-2, enhances endothelial cell barrier function and thus vascular integrity during neutrophil transendothelial migration (PubMed:23202369). May play a role in neutrophil transendothelial migration, probably when associated with CD177 (PubMed:22266279).

Gene Wiki entry for PRTN3 Gene

Additional gene information for PRTN3 Gene

No data available for Entrez Gene Summary , CIViC summary , Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for PRTN3 Gene

Genomics for PRTN3 Gene

GeneHancer (GH) Regulatory Elements for PRTN3 Gene

Promoters and enhancers for PRTN3 Gene
GeneHancer (GH) Identifier GH Type GH
GH Sources Gene Association Score Total Score TSS distance (kb) Number of Genes Away Size (kb) Transcription Factor
Binding Sites
Gene Targets
GH19J000840 Promoter/Enhancer 1.3 EPDnew Ensembl 650.7 +0.0 41 0.8 HDGF PKNOX1 ARNT NFRKB ZNF366 ZFP91 EGR2 SMARCA5 ZBTB2 NCOA1 PRTN3 ELANE
GH19J000839 Enhancer 0.4 Ensembl 650.7 -1.6 -1559 0.8 GLIS1 CEBPB PRTN3 GC19M000833
GH19J000747 Promoter/Enhancer 2.1 EPDnew FANTOM5 Ensembl ENCODE dbSUPER 13.3 -89.0 -89045 9.4 PKNOX1 ATF1 FOXA2 ARID4B SIN3A ZBTB7B BRCA1 ZNF143 FOS SP5 MISP PRTN3 ELANE CFD FSTL3 PLPP2 TPGS1 GC19P000749 GC19P000763 PALM
GH19J000823 Promoter/Enhancer 1.7 EPDnew FANTOM5 ENCODE 12.4 -17.2 -17248 0.5 HDAC1 ATF1 RB1 NFRKB POLR2B ZNF766 ATF7 CREM SMAD5 ZBTB2 AZU1 PLPPR3 GC19P000823 PRTN3 ELANE PRSS57 BSG PTBP1
- Elite GeneHancer and/or Elite GeneHancer-gene association Download GeneHancer data from 2017 publication | Request up-to-date GeneHancer data (full dataset)

GeneHancers around PRTN3 on UCSC Golden Path with GeneCards custom track

Top Transcription factor binding sites by QIAGEN in the PRTN3 gene promoter:
  • AML1a

Genomic Locations for PRTN3 Gene

Genomic Locations for PRTN3 Gene
7,216 bases
Plus strand
7,213 bases
Plus strand

Genomic View for PRTN3 Gene

Genes around PRTN3 on UCSC Golden Path with GeneCards custom track

Cytogenetic band:
PRTN3 Gene in genomic location: bands according to Ensembl, locations according to GeneLoc (and/or Entrez Gene and/or Ensembl if different)
Genomic Location for PRTN3 Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for PRTN3 Gene

Proteins for PRTN3 Gene

  • Protein details for PRTN3 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Protein Accession:
    Secondary Accessions:
    • P15637
    • P18078
    • Q4VB08
    • Q4VB09
    • Q6LBM7
    • Q6LBN2
    • Q9UD25
    • Q9UQD8

    Protein attributes for PRTN3 Gene

    256 amino acids
    Molecular mass:
    27807 Da
    Quaternary structure:
    • May form dimers (PubMed:28240246). Interacts with CD177; the interaction tethers PRTN3 to the cell surface; the interaction is direct (PubMed:17244676, PubMed:28240246).
    • Sequence=AAA36342.1; Type=Frameshift; Positions=34, 39; Evidence={ECO:0000305}; Sequence=CAA39598.1; Type=Erroneous initiation; Evidence={ECO:0000305};

    Three dimensional structures from OCA and Proteopedia for PRTN3 Gene

neXtProt entry for PRTN3 Gene

Post-translational modifications for PRTN3 Gene

  • Glycosylation at posLast=174174 and Asn129

Other Protein References for PRTN3 Gene

ENSEMBL proteins:
REFSEQ proteins:

No data available for DME Specific Peptides for PRTN3 Gene

Domains & Families for PRTN3 Gene

Gene Families for PRTN3 Gene

Human Protein Atlas (HPA):
  • Candidate cardiovascular disease genes
  • Enzymes
  • Plasma proteins
  • Predicted intracellular proteins
  • Predicted secreted proteins

Suggested Antigen Peptide Sequences for PRTN3 Gene

Graphical View of Domain Structure for InterPro Entry



  • Belongs to the peptidase S1 family. Elastase subfamily.
  • Belongs to the peptidase S1 family. Elastase subfamily.
genes like me logo Genes that share domains with PRTN3: view

Function for PRTN3 Gene

Molecular function for PRTN3 Gene

UniProtKB/Swiss-Prot Function:
Serine protease that degrades elastin, fibronectin, laminin, vitronectin, and collagen types I, III, and IV (in vitro) (PubMed:3198760, PubMed:2033050, PubMed:28240246). By cleaving and activating receptor F2RL1/PAR-2, enhances endothelial cell barrier function and thus vascular integrity during neutrophil transendothelial migration (PubMed:23202369). May play a role in neutrophil transendothelial migration, probably when associated with CD177 (PubMed:22266279).
UniProtKB/Swiss-Prot CatalyticActivity:
Hydrolysis of proteins, including elastin, by preferential cleavage: -Ala- -Xaa- > -Val- -Xaa-.
UniProtKB/Swiss-Prot EnzymeRegulation:
Inhibited by phenylmethanesulfonyl fluoride (PMSF) and diisopropyl fluorophosphate (DFP).
UniProtKB/Swiss-Prot BiophysicochemicalProperties:
pH dependence: Optimum pH is 7.4. {ECO:0000269 PubMed:3198760};
UniProtKB/Swiss-Prot Induction:
Induced during CSF3/G-CSF-mediated neutrophil differentiation.
GENATLAS Biochemistry:
proteinase 3,myeloblastin,Wegener autoantigen,responsive to CSF3,stimulated by SPI1,MYB,inducing factor independent growth of hematopoietic cells

Enzyme Numbers (IUBMB) for PRTN3 Gene

Phenotypes From GWAS Catalog for PRTN3 Gene

Gene Ontology (GO) - Molecular Function for PRTN3 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0004252 serine-type endopeptidase activity IMP 22266279
GO:0005102 signaling receptor binding IPI 17244676
GO:0005515 protein binding IPI 15527767
GO:0008233 peptidase activity IEA --
GO:0008236 serine-type peptidase activity IEA,TAS 2679910
genes like me logo Genes that share ontologies with PRTN3: view
genes like me logo Genes that share phenotypes with PRTN3: view

Human Phenotype Ontology for PRTN3 Gene

HPO Id HPO Name Alternative Ids Definition Synonyms

Animal Models for PRTN3 Gene

MGI Knock Outs for PRTN3:

Animal Model Products

CRISPR Products

Inhibitory RNA Products

  • Search GeneCopoeia for shRNA, lentivirus and/or AAV clone products for PRTN3

No data available for miRNA , Transcription Factor Targets and HOMER Transcription for PRTN3 Gene

Localization for PRTN3 Gene

Subcellular locations from UniProtKB/Swiss-Prot for PRTN3 Gene

Cytoplasmic granule. Secreted. Cell membrane; Peripheral membrane protein; Extracellular side. Membrane raft; Peripheral membrane protein; Extracellular side. Note=Localizes predominantly to azurophil granules (primary secretory granules) in neutrophils (PubMed:2033050, PubMed:3198760, PubMed:7897245, PubMed:18462208). Secreted upon neutrophil stimulation by TNF-alpha, 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}.

Subcellular locations from

Extracellular space Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi Apparatus Nucleus Mitochondrion 0 1 2 3 4 5 Confidence
COMPARTMENTS Subcellular localization image for PRTN3 gene
Compartment Confidence
plasma membrane 5
extracellular 5
cytosol 5
lysosome 5
cytoskeleton 2
nucleus 2

Gene Ontology (GO) - Cellular Components for PRTN3 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005576 extracellular region TAS --
GO:0005615 extracellular space HDA,IBA --
GO:0005829 cytosol TAS --
GO:0005886 plasma membrane TAS --
GO:0016020 membrane IEA --
genes like me logo Genes that share ontologies with PRTN3: view

No data available for Subcellular locations from the Human Protein Atlas (HPA) for PRTN3 Gene

Pathways & Interactions for PRTN3 Gene

genes like me logo Genes that share pathways with PRTN3: view

Gene Ontology (GO) - Biological Process for PRTN3 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0006508 proteolysis IEA,IMP 22266279
GO:0006509 membrane protein ectodomain proteolysis IMP 23202369
GO:0006909 phagocytosis IBA --
GO:0007596 blood coagulation TAS --
GO:0008284 positive regulation of cell proliferation TAS 2598267
genes like me logo Genes that share ontologies with PRTN3: view

No data available for SIGNOR curated interactions for PRTN3 Gene

Drugs & Compounds for PRTN3 Gene

(28) Drugs for PRTN3 Gene - From: DrugBank, DGIdb, and Novoseek

Name Status Disease Links Group Role Mechanism of Action Clinical Trials
Elafin Investigational Pharma Target 2
Gabexate mesylate Pharma Trypsin-like serine proteinases inhibitor 0

(18) Additional Compounds for PRTN3 Gene - From: Novoseek

Name Synonyms Role CAS Number PubChem IDs PubMed IDs
genes like me logo Genes that share compounds with PRTN3: view

Transcripts for PRTN3 Gene

mRNA/cDNA for PRTN3 Gene

(2) REFSEQ mRNAs :
(10) Additional mRNA sequences :
(27) Selected AceView cDNA sequences:
(2) Ensembl transcripts including schematic representations, and UCSC links where relevant :

Unigene Clusters for PRTN3 Gene

Proteinase 3:
Representative Sequences:

CRISPR Products

Inhibitory RNA Products

  • Search GeneCopoeia for shRNA, lentivirus and/or AAV clone products for PRTN3

Alternative Splicing Database (ASD) splice patterns (SP) for PRTN3 Gene

No ASD Table

Relevant External Links for PRTN3 Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for PRTN3 Gene

mRNA expression in normal human tissues from GTEx, Illumina, BioGPS, and CGAP SAGE for PRTN3 Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

mRNA differential expression in normal tissues according to GTEx for PRTN3 Gene

This gene is overexpressed in Whole Blood (x41.9) and Spleen (x4.8).

Protein differential expression in normal tissues from HIPED for PRTN3 Gene

This gene is overexpressed in Neutrophil (22.5), Lavage (7.7), Cervix (6.6), Monocytes (6.3), and Spleen (6.2).

Integrated Proteomics: protein expression in normal tissues and cell lines from ProteomicsDB and MOPED for PRTN3 Gene

Protein tissue co-expression partners for PRTN3 Gene

- Elite partner

NURSA nuclear receptor signaling pathways regulating expression of PRTN3 Gene:


SOURCE GeneReport for Unigene cluster for PRTN3 Gene:


mRNA Expression by UniProt/SwissProt for PRTN3 Gene:

Tissue specificity: Expressed in polymorphonuclear leukocytes (at protein level) (PubMed:2033050, PubMed:7897245, PubMed:3198760). Expressed in neutrophils (at protein level) (PubMed:28240246, PubMed:18462208, PubMed:21193407, PubMed:22266279, PubMed:17244676). Expressed in differentiating neutrophils (PubMed:18462208).

Evidence on tissue expression from TISSUES for PRTN3 Gene

  • Blood(4.6)
  • Bone marrow(3.4)
  • Lung(2.7)
  • Kidney(2.6)
  • Spleen(2.4)
  • Heart(2.1)
  • Skin(2)
  • Nervous system(2)

Phenotype-based relationships between genes and organs from Gene ORGANizer for PRTN3 Gene

Germ Layers:
  • ectoderm
  • endoderm
  • mesoderm
  • cardiovascular
  • digestive
  • endocrine
  • immune
  • integumentary
  • nervous
  • respiratory
  • skeleton
  • urinary
Head and neck:
  • brain
  • ear
  • eye
  • face
  • head
  • larynx
  • mouth
  • neck
  • nose
  • pituitary gland
  • sinus
  • skull
  • bronchus
  • esophagus
  • heart
  • lung
  • trachea
  • kidney
  • stomach
  • ankle
  • digit
  • elbow
  • finger
  • foot
  • hand
  • hip
  • knee
  • lower limb
  • shoulder
  • toe
  • upper limb
  • wrist
  • blood
  • blood vessel
  • coagulation system
  • peripheral nerve
  • peripheral nervous system
  • red blood cell
  • skin
  • white blood cell
genes like me logo Genes that share expression patterns with PRTN3: view

Orthologs for PRTN3 Gene

This gene was present in the common ancestor of animals.

Orthologs for PRTN3 Gene

Organism Taxonomy Gene Similarity Type Details
(Pan troglodytes)
Mammalia PRTN3 34
  • 95 (a)
(Bos Taurus)
Mammalia PRTN3 34 33
  • 75.3 (n)
(Mus musculus)
Mammalia Prtn3 16 34 33
  • 73.19 (n)
(Rattus norvegicus)
Mammalia Prtn3 33
  • 70.96 (n)
(Monodelphis domestica)
Mammalia PRTN3 34
  • 53 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia prtn3 33
  • 55.61 (n)
African malaria mosquito
(Anopheles gambiae)
Insecta AgaP_AGAP007142 33
  • 47.76 (n)
Species where no ortholog for PRTN3 was found in the sources mined by GeneCards:
  • A. gosspyii yeast (Ashbya gossypii)
  • Actinobacteria (Mycobacterium tuberculosis)
  • African clawed frog (Xenopus laevis)
  • Alicante grape (Vitis vinifera)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • baker's yeast (Saccharomyces cerevisiae)
  • barley (Hordeum vulgare)
  • beta proteobacteria (Neisseria meningitidis)
  • bread mold (Neurospora crassa)
  • chicken (Gallus gallus)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • corn (Zea mays)
  • dog (Canis familiaris)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • fission yeast (Schizosaccharomyces pombe)
  • fruit fly (Drosophila melanogaster)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • K. lactis yeast (Kluyveromyces lactis)
  • lizard (Anolis carolinensis)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • platypus (Ornithorhynchus anatinus)
  • rainbow trout (Oncorhynchus mykiss)
  • rice (Oryza sativa)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea squirt (Ciona intestinalis)
  • sea squirt (Ciona savignyi)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • soybean (Glycine max)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • thale cress (Arabidopsis thaliana)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • wheat (Triticum aestivum)
  • worm (Caenorhabditis elegans)
  • zebrafish (Danio rerio)

Evolution for PRTN3 Gene

Gene Tree for PRTN3 (if available)
Gene Tree for PRTN3 (if available)
Evolutionary constrained regions (ECRs) for PRTN3: view image

Paralogs for PRTN3 Gene

Paralogs for PRTN3 Gene

genes like me logo Genes that share paralogs with PRTN3: view

Variants for PRTN3 Gene

Sequence variations from dbSNP and Humsavar for PRTN3 Gene

SNP ID Clin Chr 19 pos Variation AA Info Type
rs1000024445 -- 841,279(+) C/T intron_variant
rs1001347644 -- 844,276(+) T/C intron_variant
rs1001619269 -- 839,741(+) G/A upstream_transcript_variant
rs1001960501 -- 843,436(+) T/C intron_variant
rs1002029059 -- 839,586(+) G/A upstream_transcript_variant

Structural Variations from Database of Genomic Variants (DGV) for PRTN3 Gene

Variant ID Type Subtype PubMed ID
dgv134n111 CNV deletion 26073780
dgv1679n106 CNV deletion 24896259
esv2717797 CNV deletion 23290073
esv2717798 CNV deletion 23290073
nsv1112307 CNV deletion 24896259
nsv138320 CNV deletion 16902084
nsv458321 CNV loss 19166990
nsv470101 CNV loss 18288195
nsv578167 CNV loss 21841781
nsv578172 CNV loss 21841781
nsv953938 CNV deletion 24416366

Variation tolerance for PRTN3 Gene

Residual Variation Intolerance Score: 76.7% of all genes are more intolerant (likely to be disease-causing)
Gene Damage Index Score: 7.38; 81.73% of all genes are more intolerant (likely to be disease-causing)

Additional Variant Information for PRTN3 Gene

SNPedia medical, phenotypic, and genealogical associations of SNPs for

SNP Genotyping and Copy Number Assay Products

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for PRTN3 Gene

Disorders for PRTN3 Gene

MalaCards: The human disease database

(49) MalaCards diseases for PRTN3 Gene - From: Orphanet, DISEASES, Novoseek, and GeneCards

Disorder Aliases PubMed IDs
wegener granulomatosis
  • wg
  • angiitis
rapidly progressive glomerulonephritis
  • crescentic glomerulonephritis
churg-strauss syndrome
  • allergic granulomatosis angiitis
anca-associated vasculitis
  • aav
- elite association - COSMIC cancer census association via MalaCards
Search PRTN3 in MalaCards View complete list of genes associated with diseases


  • Note=Is the major autoantigen in anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (Wegeners 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 cytoplasmatic 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}.

Additional Disease Information for PRTN3

Genetic Association Database
Human Genome Epidemiology Navigator
ATLAS of Genetics and Cytogenetics in Oncology and Haematology
genes like me logo Genes that share disorders with PRTN3: view

No data available for Genatlas for PRTN3 Gene

Publications for PRTN3 Gene

  1. Cloning of cDNA for proteinase 3: a serine protease, antibiotic, and autoantigen from human neutrophils. (PMID: 2258701) Campanelli D … Gabay JE (The Journal of experimental medicine 1990) 2 3 4 22 58
  2. Neutrophil surface presentation of the anti-neutrophil cytoplasmic antibody-antigen proteinase 3 depends on N-terminal processing. (PMID: 18462208) von Vietinghoff S … Kettritz R (Clinical and experimental immunology 2008) 3 4 22 58
  3. Increased neutrophil membrane expression and plasma level of proteinase 3 in systemic vasculitis are not a consequence of the - 564 A/G promotor polymorphism. (PMID: 16792675) Abdgawad M … Segelmark M (Clinical and experimental immunology 2006) 3 22 44 58
  4. Proteinase 3 gene polymorphisms and Wegener's granulomatosis. (PMID: 11115080) Gencik M … Fricke H (Kidney international 2000) 3 22 44 58
  5. The crystal structure of PR3, a neutrophil serine proteinase antigen of Wegener's granulomatosis antibodies. (PMID: 8757293) Fujinaga M … James MN (Journal of molecular biology 1996) 3 4 22 58

Products for PRTN3 Gene

Sources for PRTN3 Gene

Loading form....