PRTN3
Myeloblastin
Also known as: ACPA, AGP7, C-ANCA, MBT, P29, PR-3, PRTN3_HUMAN
Cross-references: UniProt · Ensembl · Human Protein Atlas · GeneCards · NCBI Gene
Protein identityUniProt · HPA
- UniProt accession
- P24158
- Gene
- PRTN3
- Ensembl
- ENSG00000196415
- Chromosome
- 19
- Canonical length
- 256 aa
- Protein class
- Candidate cardiovascular disease genes, Disease related genes, Enzymes, Plasma proteins, Potential drug targets, Predicted intracellular proteins, Predicted secreted proteins
- Subcellular location
- Vesicles,Cytosol
- Secretome location
- Secreted to blood
OverviewNCBI Gene
Enables enzyme binding activity; serine-type endopeptidase activity; and signaling receptor binding activity. Involved in several processes, including mature conventional dendritic cell differentiation; neutrophil extravasation; and positive regulation of GTPase activity. Located in azurophil granule lumen; cytosol; and plasma membrane raft. [provided by Alliance of Genome Resources, Jul 2025]
Canonical amino-acid sequenceUniProt
256 residues, UniProt reviewed canonical sequence.
>P24158|PRTN3
1 MAHRPPSPAL ASVLLALLLS GAARAAEIVG GHEAQPHSRP YMASLQMRGN PGSHFCGGTL
61 IHPSFVLTAA HCLRDIPQRL VNVVLGAHNV RTQEPTQQHF SVAQVFLNNY DAENKLNDVL
121 LIQLSSPANL SASVATVQLP QQDQPVPHGT QCLAMGWGRV GAHDPPAQVL QELNVTVVTF
181 FCRPHNICTF VPRRKAGICF GDSGGPLICD GIIQGIDSFV IWGCATRLFP DFFTRVALYV
241 DWIRSTLRRV EAKGRPLocalizationUniProt · AlphaFold · HPA
Whether an antibody against PRTN3 can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.
- Antibody reachability
- Cell surface
- Secreted
- No
- Transmembrane segments
- 0
- Mean surface accessibility (rSASA)
- 0.28
- Highest tissue expression
- 1,978 nTPM
Expression across tissuesHPA
Tissue
- bone marrow: 1,978 nTPM
- spleen: 38 nTPM
- lung: 12 nTPM
- thymus: 6.7 nTPM
- cerebral cortex: 3.6 nTPM
- liver: 2.7 nTPM
Single-cell type
- neutrophil progenitors: 339 nCPM
- monocyte progenitors: 320 nCPM
- erythrocytes: 5.9 nCPM
- hematopoietic stem cells: 1 nCPM
- kupffer cells: 1 nCPM
- brain excitatory neurons: 0.7 nCPM
Immune cell
- total PBMC: 9.9 nTPM
- neutrophil: 2 nTPM
- gdT-cell: 0.2 nTPM
- basophil: 0 nTPM
- classical monocyte: 0 nTPM
- eosinophil: 0 nTPM
Brain region
- cerebral cortex: 1.4 nTPM
- basal ganglia: 1.2 nTPM
- hypothalamus: 0.9 nTPM
- amygdala: 0.8 nTPM
- hippocampal formation: 0.6 nTPM
- midbrain: 0.6 nTPM
DiseaseUniProt · ClinVar · IEDB · PubMed
Four sources answering four different questions about PRTN3.
Disease | ImmuneIEDB
Conditions an epitope on PRTN3 was assayed in.
- granulomatosis with polyangiitis B and T cell
- systemic scleroderma B cell
- autoimmune vasculitis B cell
- Goodpasture syndrome B cell
- systemic lupus erythematosus B cell
- chronic myeloid leukemia T cell
- acute myeloid leukemia T cell
- viral infectious disease T cell
- multiple myeloma T cell
- chronic myelogenous leukemia T cell
- leukemia T cell
- acute lymphoblastic leukemia T cell
- cancer T cell
- acute myeloid leukemia with minimal differentiation T cell
Disease | AutoantibodyPubMed
Conditions in which antibodies against PRTN3 are reported. Each links to that disease's full target list.
- Granulomatosis with Polyangiitis 219
- Vasculitis 93
- Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis 86
- Glomerulonephritis 54
- Colitis, Ulcerative 25
- Crohn Disease 11
- Lupus Erythematosus, Systemic 11
- Arthritis, Rheumatoid 10
- Inflammatory Bowel Diseases 9
- Kidney Diseases 9
- Kidney Failure, Chronic 9
- Sjogren's Syndrome 8
- Anti-Glomerular Basement Membrane Disease 7
- Graves Disease 6
- Mixed Connective Tissue Disease 6
- Acute Kidney Injury 5
- Lung Diseases, Interstitial 5
- COVID-19 4
- Glomerulonephritis, IGA 4
- Lupus Nephritis 4
Showing 20 of 52 — disease pages carrying at least 10 antigens.
ReferencesPubMed · IEDB
Publications for PRTN3 from three distinct lines of evidence, kept separate because they answer different questions: whether antibodies are directed at the protein, whether a B-cell epitope has been mapped on it, and whether a T-cell epitope has. Each is labelled with its source.
Reference: AutoantibodyPubMed
552 publications
- ANCA-associated vasculitis.
2020 · Nat Rev Dis Primers · RCR 40.6 · 718 citations - Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients.
1999 · Arthritis Rheum · RCR 17.5 · 520 citations - Alternative complement pathway in the pathogenesis of disease mediated by anti-neutrophil cytoplasmic autoantibodies.
2007 · Am J Pathol · RCR 11.4 · 462 citations - Predictors of relapse and treatment resistance in antineutrophil cytoplasmic antibody-associated small-vessel vasculitis.
2005 · Ann Intern Med · RCR 10.6 · 371 citations - Risk factors for relapse of antineutrophil cytoplasmic antibody-associated vasculitis.
2012 · Arthritis Rheum · RCR 10.1 · 290 citations
Show 20 more of 552 total
- ANCA and associated diseases: immunodiagnostic and pathogenetic aspects.
1993 · Clin Exp Immunol · RCR 7.4 · 206 citations - A new type of perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) in active ulcerative colitis but not in Crohn's disease.
1990 · Immunobiology · RCR 7.3 · 217 citations - Prevalence of antineutrophil cytoplasmic antibodies in a large inception cohort of patients with connective tissue disease.
1997 · Ann Intern Med · RCR 7.3 · 192 citations - Anti-neutrophil cytoplasmic antibodies engage and activate human neutrophils via Fc gamma RIIa.
1994 · J Immunol · RCR 6.6 · 251 citations - Autoimmunity is triggered by cPR-3(105-201), a protein complementary to human autoantigen proteinase-3.
2004 · Nat Med · RCR 6.1 · 253 citations - Factors Determining the Clinical Utility of Serial Measurements of Antineutrophil Cytoplasmic Antibodies Targeting Proteinase 3.
2016 · Arthritis Rheumatol · RCR 5.8 · 129 citations - The syndrome of lung hemorrhage and nephritis is usually an ANCA-associated condition.
1996 · Arch Intern Med · RCR 5.6 · 132 citations - Antiproteinase 3 antineutrophil cytoplasmic antibodies and disease activity in Wegener granulomatosis.
2007 · Ann Intern Med · RCR 5.4 · 191 citations - Contaminated cocaine and antineutrophil cytoplasmic antibody-associated disease.
2011 · Clin J Am Soc Nephrol · RCR 5.3 · 133 citations - Protein microarrays with carbon nanotubes as multicolor Raman labels.
2008 · Nat Biotechnol · RCR 5.1 · 198 citations - Trojan horses: drug culprits associated with antineutrophil cytoplasmic autoantibody (ANCA) vasculitis.
2014 · Curr Opin Rheumatol · RCR 4.9 · 121 citations - Antineutrophil cytoplasmic antibodies and associated diseases: a review of the clinical and laboratory features.
2000 · Kidney Int · RCR 4.8 · 174 citations - Human endothelial cells express proteinase 3, the target antigen of anticytoplasmic antibodies in Wegener's granulomatosis.
1993 · Blood · RCR 4.6 · 163 citations - Remote self-collection of capillary blood using upper arm devices for autoantibody analysis in patients with immune-mediated inflammatory rheumatic diseases.
2022 · RMD Open · RCR 4.6 · 39 citations - The pathologic spectrum of pulmonary lesions in patients with anti-neutrophil cytoplasmic autoantibodies specific for anti-proteinase 3 and anti-myeloperoxidase.
1995 · Am J Clin Pathol · RCR 4.4 · 114 citations - The PiZ gene of alpha 1-antitrypsin as a determinant of outcome in PR3-ANCA-positive vasculitis.
1995 · Kidney Int · RCR 4.3 · 135 citations - Antineutrophil cytoplasmic antibodies (ANCA) directed against cathepsin G in ulcerative colitis, Crohn's disease and primary sclerosing cholangitis.
1992 · Clin Exp Immunol · RCR 4.3 · 135 citations - Antineutrophil cytoplasmic autoantibodies against the murine homolog of proteinase 3 (Wegener autoantigen) are pathogenic in vivo.
2004 · Blood · RCR 4.3 · 181 citations - Antineutrophil cytoplasmic antibody-associated vasculitides: is it time to split up the group?
2013 · Ann Rheum Dis · RCR 4.2 · 116 citations - Subacute bacterial endocarditis with positive cytoplasmic antineutrophil cytoplasmic antibodies and anti-proteinase 3 antibodies.
2000 · Arthritis Rheum · RCR 4.1 · 137 citations
Reference: B cellIEDB
7 publications
- Anti-neutrophil cytoplasmic antibodies (ANCA) from patients with systemic vasculitis recognize restricted epitopes of proteinase 3 involving the catalytic site.
2001 · Clin Exp Immunol · RCR 1.2 · 48 citations - Impact of peptides on the recognition of HLA class I molecules by human HLA antibodies.
2005 · J Immunol · RCR 1.1 · 49 citations - Computational analysis of high-density peptide microarray data with application from systemic sclerosis to multiple sclerosis.
2012 · Autoimmun Rev · RCR 1 · 35 citations - Antineutrophil cytoplasmic antibodies to proteinase 3 in Wegener's granulomatosis: epitope analysis using synthetic peptides.
2001 · Kidney Int · RCR 0.9 · 40 citations - A population of autoantibodies against a centromere-associated protein A major epitope motif cross-reacts with related cryptic epitopes on other nuclear autoantigens and on the Epstein-Barr nuclear antigen 1.
2001 · J Mol Med (Berl) · RCR 0.6 · 28 citations
Show 2 more
- Anti-neutrophil cytoplasmic antibodies target sequential functional proteinase 3 epitopes in the sera of patients with Wegener’s granulomatosis.
2010 · Clin Exp Immunol · RCR 0.3 · 11 citations - Defensin Interactions in Relation to Monoclonal and Disease-Related Proteinase 3 Antibodies Binding at the Catalytic Site.
2023 · Antibodies (Basel)
Reference: T cellIEDB
13 publications
- PR1 peptide vaccine induces specific immunity with clinical responses in myeloid malignancies.
2017 · Leukemia · RCR 2.9 · 88 citations - Expression of the CTLA-4 ligand CD86 on plasmacytoid dendritic cells (pDC) predicts risk of disease recurrence after treatment discontinuation in CML.
2017 · Leukemia · RCR 2.5 · 77 citations - Selective depletion of donor alloreactive T cells without loss of antiviral or antileukemic responses.
2003 · Blood · RCR 2.4 · 122 citations - Cell-mediated autoimmunity in patients with Wegener's granulomatosis (WG).
1995 · Clin Exp Immunol · RCR 1.5 · 58 citations - ANCA patients have T cells responsive to complementary PR-3 antigen.
2008 · Kidney Int · RCR 1.1 · 43 citations
Show 8 more
- A phase I/II clinical trial of autologous cytokine-induced killer cells as adjuvant immunotherapy for acute and chronic myeloid leukemia in clinical remission.
2012 · Cytotherapy · RCR 1 · 42 citations - PR1-specific T cells are associated with unmaintained cytogenetic remission of chronic myelogenous leukemia after interferon withdrawal.
2010 · PLoS One · RCR 0.6 · 29 citations - CD4(+)and CD8(+)T-cell reactions against leukemia-associated- or minor-histocompatibility-antigens in AML-patients after allogeneic SCT.
2014 · Immunobiology · RCR 0.6 · 22 citations - Frequency of proteinase 3 (PR3)-specific autoreactive T cells determined by cytokine flow cytometry in Wegener's granulomatosis.
2004 · J Autoimmun · RCR 0.5 · 21 citations - Lymphodepletion is permissive to the development of spontaneous T-cell responses to the self-antigen PR1 early after allogeneic stem cell transplantation and in patients with acute myeloid leukemia undergoing WT1 peptide vaccination following chemotherapy.
2012 · Cancer Immunol Immunother · RCR 0.4 · 15 citations - PR1-specific cytotoxic T lymphocytes are relatively frequent in umbilical cord blood and can be effectively expanded to target myeloid leukemia.
2016 · Cytotherapy · RCR 0.3 · 10 citations - Targeting the Leukemia Antigen PR1 with Immunotherapy for the Treatment of Multiple Myeloma.
2018 · Clin Cancer Res · RCR 0.1 · 4 citations - HLA class I-restricted T cell epitopes isolated and identified from myeloid leukemia cells.
2019 · Sci Rep · RCR 0.1 · 3 citations
Sources: PubMed — antigen-level antibody evidence from a custom retrieval. Records matching a controlled set of autoantibody terms (the MeSH descriptors Autoantibodies and Autoantigens, with title and abstract term variants) were obtained through NCBI E-utilities, and their titles and abstracts parsed for constructions that direct an antibody at a named protein rather than for co-occurrence. Captured names were resolved against UniProt nomenclature and each antigen adjudicated individually against the source text. IEDB — curated epitope assays from the Immune Epitope Database (Vita et al., Nucleic Acids Research 2019). Bibliographic records from PubMed and MeSH, U.S. National Library of Medicine; citation metrics from NIH iCite (Hutchins et al., PLoS Biology 2016). Titles link to PubMed; abstracts are not reproduced here. The NLM does not endorse this analysis.
Genetic constraint and essentialitygnomAD · DepMap
Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.
- gnomAD LOEUF (loss-of-function intolerance)
- 1.65
- gnomAD pLI
- 0
- gnomAD missense Z
- 0.15
- DepMap mean gene effect
- -0.02
- DepMap dependency class
- none
Cancer expressionTCGA
Across TCGA tumor cohorts, this protein is over-expressed in roughly 3% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).
OntologyGO
Biological processes
- antimicrobial humoral response
- cell-cell junction maintenance
- collagen catabolic process
- mature conventional dendritic cell differentiation
- membrane protein ectodomain proteolysis
- negative regulation of phagocytosis
- neutrophil extravasation
- positive regulation of cell population proliferation
- positive regulation of GTPase activity
- proteolysis
Molecular functions
- enzyme binding
- serine-type endopeptidase activity
- serine-type peptidase activity
- signaling receptor binding
Cellular components
Protein domainsUniProt · Pfam · InterPro
KeywordsUniProt
InteractionsUniProt · HPA
Protein binding partners of PRTN3 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.
Antibody and autoantibody relevanceSeroatlas analysis
Seroatlas reads PRTN3 as an antibody target. Whether an autoantibody or antibody against PRTN3 could matter depends on whether native PRTN3 is physically reachable, whether the body needs it intact, and whether it acts in a disease-relevant tissue.
PRTN3 is annotated at the cell surface, where native PRTN3 is exposed to circulating antibodies and is a prime autoantibody target that could block, deplete, or overstimulate it.
Annotation status
The present source text does not explicitly label PRTN3 as an autoantigen. Seroatlas presents hypothesis context only and does not manufacture a known-serology claim.
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