Reviewed,
UniProtKB/Swiss-Prot P24158 (PRTN3_HUMAN)
Last modified
June 16, 2009.
Version 109.
History...
Clusters with 100%,
90%,
50% identity |
Documents (7) |
Third-party data |
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Names and origin
| Protein names | Recommended name: Myeloblastin EC=3.4.21.76 Alternative name(s): Leukocyte proteinase 3 Short name=PR-3 Short name=PR3 AGP7 Wegener autoantigen P29 C-ANCA antigen Neutrophil proteinase 4 Short name=NP-4 | ||||
| Gene names |
| ||||
| Organism | Homo sapiens (Human) | ||||
| Taxonomic identifier | 9606 [NCBI] | ||||
| Taxonomic lineage | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Protein attributes
| Sequence length | 256 AA. |
| Sequence status | Complete. |
| Sequence processing | The displayed sequence is further processed into a mature form. |
| Protein existence | Evidence at protein level. |
General annotation (Comments)
| Function | Polymorphonuclear leukocyte serine protease that degrades elastin, fibronectin, laminin, vitronectin, and collagen types I, III, and IV (in vitro) and causes emphysema when administered by tracheal insufflation to hamsters. |
| Catalytic activity | Hydrolysis of proteins, including elastin, by preferential cleavage: -Ala-|-Xaa- > -Val-|-Xaa-. |
| Sequence similarities | Belongs to the peptidase S1 family. Elastase subfamily. Contains 1 peptidase S1 domain. |
| Sequence caution | The sequence AAA36342.1 differs from that shown. Reason: Frameshift at positions 34 and 39. |
Ontologies
| Keywords | |
|---|---|
| Biological process | Collagen degradation |
| Coding sequence diversity | Polymorphism |
| Domain | Signal |
| Molecular function | Hydrolase Protease Serine protease |
| PTM | Disulfide bond Glycoprotein Zymogen |
| Technical term | 3D-structure Direct protein sequencing |
| Gene Ontology (GO) | |
| Biological process | collagen catabolic process Inferred from electronic annotation. Source: UniProtKB-KW positive regulation of cell proliferation Ref.10Traceable author statement. Source: ProtInc proteolysisInferred from electronic annotation. Source: InterPro |
| Cellular component | intracellular membrane-bounded organelle Inferred from direct assay. Source: HPA |
| Molecular function | protein binding Inferred from physical interaction. Source: IntAct serine-type endopeptidase activityInferred from electronic annotation. Source: InterPro |
| Complete GO annotation... | |
Sequence annotation (Features)
| Feature key | Position(s) | Length | Description | Graphical view | Feature identifier | ||||||||||||||||||||||||||||||||||||||||||||
Molecule processing | |||||||||||||||||||||||||||||||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Signal peptide | 1 – 25 | 25 | |||||||||||||||||||||||||||||||||||||||||||||||
| Propeptide | 26 – 27 | 2 | Ref.9 Ref.11 Ref.12 Ref.13 Ref.14 Ref.15 Ref.17 Ref.16 Ref.19 | PRO_0000027707 | |||||||||||||||||||||||||||||||||||||||||||||
| Chain | 28 – 248 | 221 | Myeloblastin | PRO_0000027708 | |||||||||||||||||||||||||||||||||||||||||||||
| Propeptide | 249 – 256 | 8 | PRO_0000027709 | ||||||||||||||||||||||||||||||||||||||||||||||
Regions | |||||||||||||||||||||||||||||||||||||||||||||||||
| Domain | 28 – 248 | 221 | Peptidase S1 | ||||||||||||||||||||||||||||||||||||||||||||||
Sites | |||||||||||||||||||||||||||||||||||||||||||||||||
| Active site | 71 | 1 | Charge relay system | ||||||||||||||||||||||||||||||||||||||||||||||
| Active site | 118 | 1 | Charge relay system | ||||||||||||||||||||||||||||||||||||||||||||||
| Active site | 203 | 1 | Charge relay system | ||||||||||||||||||||||||||||||||||||||||||||||
Amino acid modifications | |||||||||||||||||||||||||||||||||||||||||||||||||
| Glycosylation | 129 | 1 | N-linked (GlcNAc...) Potential | ||||||||||||||||||||||||||||||||||||||||||||||
| Glycosylation | 174 | 1 | N-linked (GlcNAc...) | ||||||||||||||||||||||||||||||||||||||||||||||
| Disulfide bond | 56 ↔ 72 | ||||||||||||||||||||||||||||||||||||||||||||||||
| Disulfide bond | 152 ↔ 209 | ||||||||||||||||||||||||||||||||||||||||||||||||
| Disulfide bond | 182 ↔ 188 | ||||||||||||||||||||||||||||||||||||||||||||||||
| Disulfide bond | 199 ↔ 224 | ||||||||||||||||||||||||||||||||||||||||||||||||
Natural variations | |||||||||||||||||||||||||||||||||||||||||||||||||
| Natural variant | 119 | 1 | V → I: dbSNP rs351111. Ref.11 Ref.1 Ref.7 Ref.10 | VAR_011691 | |||||||||||||||||||||||||||||||||||||||||||||
| Natural variant | 135 | 1 | A → T: dbSNP rs1042281. Ref.11 Ref.1 Ref.10 | VAR_011713 | |||||||||||||||||||||||||||||||||||||||||||||
| Natural variant | 136 | 1 | T → S: dbSNP rs1042282. Ref.11 Ref.1 Ref.10 | VAR_011714 | |||||||||||||||||||||||||||||||||||||||||||||
Experimental info | |||||||||||||||||||||||||||||||||||||||||||||||||
| Sequence conflict | 2 | 1 | A → R in CAA39203. Ref.9 | ||||||||||||||||||||||||||||||||||||||||||||||
| Sequence conflict | 38 | 1 | S → I AA sequence Ref.19 | ||||||||||||||||||||||||||||||||||||||||||||||
| Sequence conflict | 40 | 1 | P → PI AA sequence Ref.19 | ||||||||||||||||||||||||||||||||||||||||||||||
| Sequence conflict | 46 | 1 | Q → E AA sequence Ref.13 | ||||||||||||||||||||||||||||||||||||||||||||||
| Sequence conflict | 46 | 1 | Q → E AA sequence Ref.17 | ||||||||||||||||||||||||||||||||||||||||||||||
| Sequence conflict | 48 | 1 | R → A AA sequence Ref.14 | ||||||||||||||||||||||||||||||||||||||||||||||
| Sequence conflict | 64 | 1 | S → D AA sequence Ref.12 | ||||||||||||||||||||||||||||||||||||||||||||||
| Sequence conflict | 70 | 1 | A → P in AAA59558. Ref.1 | ||||||||||||||||||||||||||||||||||||||||||||||
| Sequence conflict | 255 | 1 | Missing in CAA39203. Ref.9 | ||||||||||||||||||||||||||||||||||||||||||||||
Secondary structure | |||||||||||||||||||||||||||||||||||||||||||||||||
Helix Strand Turn | |||||||||||||||||||||||||||||||||||||||||||||||||
| Beta strand | 42 – 47 | 6 | |||||||||||||||||||||||||||||||||||||||||||||||
| Beta strand | 56 – 62 | 7 | |||||||||||||||||||||||||||||||||||||||||||||||
| Beta strand | 65 – 68 | 4 | |||||||||||||||||||||||||||||||||||||||||||||||
| Helix | 70 – 73 | 4 | |||||||||||||||||||||||||||||||||||||||||||||||
| Beta strand | 74 – 76 | 3 | |||||||||||||||||||||||||||||||||||||||||||||||
| Helix | 78 – 80 | 3 | |||||||||||||||||||||||||||||||||||||||||||||||
| Beta strand | 81 – 86 | 6 | |||||||||||||||||||||||||||||||||||||||||||||||
| Beta strand | 98 – 107 | 10 | |||||||||||||||||||||||||||||||||||||||||||||||
| Turn | 112 – 115 | 4 | |||||||||||||||||||||||||||||||||||||||||||||||
| Beta strand | 120 – 126 | 7 | |||||||||||||||||||||||||||||||||||||||||||||||
| Beta strand | 151 – 160 | 10 | |||||||||||||||||||||||||||||||||||||||||||||||
| Beta strand | 162 – 164 | 3 | |||||||||||||||||||||||||||||||||||||||||||||||
| Beta strand | 171 – 178 | 8 | |||||||||||||||||||||||||||||||||||||||||||||||
| Beta strand | 186 – 190 | 5 | |||||||||||||||||||||||||||||||||||||||||||||||
| Beta strand | 192 – 195 | 4 | |||||||||||||||||||||||||||||||||||||||||||||||
| Beta strand | 206 – 209 | 4 | |||||||||||||||||||||||||||||||||||||||||||||||
| Beta strand | 212 – 219 | 8 | |||||||||||||||||||||||||||||||||||||||||||||||
| Beta strand | 221 – 223 | 3 | |||||||||||||||||||||||||||||||||||||||||||||||
| Beta strand | 227 – 229 | 3 | |||||||||||||||||||||||||||||||||||||||||||||||
| Beta strand | 231 – 235 | 5 | |||||||||||||||||||||||||||||||||||||||||||||||
| Helix | 236 – 239 | 4 | |||||||||||||||||||||||||||||||||||||||||||||||
| Helix | 240 – 247 | 8 | |||||||||||||||||||||||||||||||||||||||||||||||
Sequences
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References
| « Hide 'large scale' references | |
| [1] | "Wegener autoantigen and myeloblastin are encoded by a single mRNA." Labbaye C., Musette P., Cayre Y.E. Proc. Natl. Acad. Sci. U.S.A. 88:9253-9256(1991) [PubMed: 1681549] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA], VARIANTS ILE-119; THR-135 AND SER-136. |
| [2] | "The DNA sequence and biology of human chromosome 19." Grimwood J., Gordon L.A., Olsen A.S., Terry A., Schmutz J., Lamerdin J.E., Hellsten U., Goodstein D., Couronne O., Tran-Gyamfi M., Aerts A., Altherr M., Ashworth L., Bajorek E., Black S., Branscomb E., Caenepeel S., Carrano A.V. Lucas S.M.Nature 428:529-535(2004) [PubMed: 15057824] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA]. |
| [3] | Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L., Mobarry C.M., Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J., Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S., Turner R. Venter J.C.Submitted (SEP-2005) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA], VARIANT ILE-119. |
| [4] | "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." The MGC Project Team Genome Res. 14:2121-2127(2004) [PubMed: 15489334] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA], VARIANT ILE-119. |
| [5] | "Three human elastase-like genes coordinately expressed in the myelomonocyte lineage are organized as a single genetic locus on 19pter." Zimmer M., Medcalf R.L., Fink T.M., Mattmann C., Lichter P., Jenne D.E. Proc. Natl. Acad. Sci. U.S.A. 89:8215-8219(1992) [PubMed: 1518849] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 1-20 AND 22-256. |
| [6] | "Structure, chromosomal assignment, and expression of the gene for proteinase-3. The Wegener's granulomatosis autoantigen." Sturrock A.B., Franklin K.F., Rao G., Marshall B.C., Rebentisch M.B., Lemons R.S., Hoidal J.R. J. Biol. Chem. 267:21193-21199(1992) [PubMed: 1400430] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 1-254, VARIANTS THR-135 AND SER-136. |
| [7] | "Donor-recipient polymorphism of the proteinase 3 gene: a potential target for T-cell alloresponses to myeloid leukemia." Clave E., Molldrem J., Hensel N., Raptis A., Barrett A.J. J. Immunother. 22:1-6(1999) [PubMed: 9924693] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 1-200, VARIANT ILE-119. |
| [8] | "Wegener's autoantigen and leukemia." Musette P., Labbaye C., Dorner M.H., Cayre Y.E., Casanova J.-L., Kourilsky P. Blood 77:1398-1399(1991) [PubMed: 2001463] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 1-42. |
| [9] | "Cloning of cDNA for proteinase 3: a serine protease, antibiotic, and autoantigen from human neutrophils." Campanelli D., Melchior M., Fu Y., Nakata M., Shuman H., Nathan C., Gabay J.E. J. Exp. Med. 172:1709-1715(1990) [PubMed: 2258701] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 2-256, PROTEIN SEQUENCE OF 28-71; 156-181 AND 196-219. |
| [10] | "Down-regulation of a serine protease, myeloblastin, causes growth arrest and differentiation of promyelocytic leukemia cells." Bories D., Raynal M.-C., Solomon D.H., Darzynkiewicz Z., Cayre Y.E. Cell 59:959-968(1989) [PubMed: 2598267] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 20-256, VARIANTS ILE-119; THR-135 AND SER-136. |
| [11] | "Wegener's autoantigen decoded." Jenne D.E., Tschopp J., Luedemann J., Utecht B., Gross W.L. Nature 346:520-520(1990) [PubMed: 2377228] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 20-256, PROTEIN SEQUENCE OF 28-48, VARIANTS ILE-119; THR-135 AND SER-136, IDENTITY OF WEGENER'S AUTOANTIGEN WITH PR-3. |
| [12] | "Characterization of proteinase-3 (PR-3), a neutrophil serine proteinase. Structural and functional properties." Rao N.V., Wehner N.G., Marshall B.C., Gray W.R., Gray B.H., Hoidal J.R. J. Biol. Chem. 266:9540-9548(1991) [PubMed: 2033050] [Abstract] Cited for: PROTEIN SEQUENCE OF 28-67 AND 228-244. |
| [13] | "Characterization of two azurphil granule proteases with active-site homology to neutrophil elastase." Wilde C.G., Snable J.L., Griffith J.E., Scott R.W. J. Biol. Chem. 265:2038-2041(1990) [PubMed: 2404977] [Abstract] Cited for: PROTEIN SEQUENCE OF 28-47 AND 196-219. |
| [14] | "Monoclonal antibodies specific for neutrophil proteinase 4. Production and use for isolation of the enzyme." Ohlsson K., Linder C., Rosengren M. Biol. Chem. Hoppe-Seyler 371:549-555(1990) [PubMed: 2121162] [Abstract] Cited for: PROTEIN SEQUENCE OF 28-52. |
| [15] | "The relation of 29 kD C-ANCA antigen to proteinase 3." Goldschmeding R., Dolman K.M., van den Ende M.E., van der Meer-Gerritsen C.H., Sonnenberg A., von dem Borne A.E. APMIS Suppl. 19:26-27(1990) [PubMed: 2285532] [Abstract] Cited for: PROTEIN SEQUENCE OF 28-48. |
| [16] | "Wegener's granulomatosis autoantigen is a novel neutrophil serine proteinase." Niles J.L., McCluskey R.T., Ahmad M.F., Arnaout M.A. Blood 74:1888-1893(1989) [PubMed: 2679910] [Abstract] Cited for: PROTEIN SEQUENCE OF 28-47. |
| [17] | "Antibiotic proteins of human polymorphonuclear leukocytes." Gabay J.E., Scott R.W., Campanelli D., Griffith J., Wilde C., Marra M.N., Seeger M., Nathan C.F. Proc. Natl. Acad. Sci. U.S.A. 86:5610-5614(1989) [PubMed: 2501794] [Abstract] Cited for: PROTEIN SEQUENCE OF 28-47. |
| [18] | "Use of proteinase 3 purified by reverse phase HPLC to detect autoantibodies in systemic vasculitis." Gaskin G., Kendal H., Coulthart A., Turner N., Pusey C.D. J. Immunol. Methods 180:25-33(1995) [PubMed: 7897245] [Abstract] Cited for: PROTEIN SEQUENCE OF 28-47, CATALYTIC ACTIVITY. Tissue: Neutrophil. |
| [19] | "Anti-neutrophil cytoplasm antibodies in Wegener's granulomatosis recognize an elastinolytic enzyme." Ludemann J., Utecht B., Gross W.L. J. Exp. Med. 171:357-362(1990) [PubMed: 1688612] [Abstract] Cited for: PROTEIN SEQUENCE OF 28-43. |
| [20] | "A novel form of dipeptidylpeptidase IV found in human serum. Isolation, characterization, and comparison with T lymphocyte membrane dipeptidylpeptidase IV (CD26)." Duke-Cohan J.S., Morimoto C., Rocker J.A., Schlossman S.F. J. Biol. Chem. 270:14107-14114(1995) [PubMed: 7539799] [Abstract] Cited for: PROTEIN SEQUENCE OF 110-121, VARIANT ILE-119. Tissue: Serum. |
| [21] | "Identity of Wegener's autoantigen (p29) with proteinase 3 and myeloblastin." Gupta S.K., Niles J.L., McCluskey R.T., Arnaout M.A. Blood 76:2162-2162(1990) [PubMed: 2242436] [Abstract] Cited for: IDENTITY OF WEGENER'S AUTOANTIGEN WITH PROTEINASE 3. |
| [22] | "The crystal structure of PR3, a neutrophil serine proteinase antigen of Wegener's granulomatosis antibodies." Fujinaga M., Charnaia M.M., Halenbeck R., Koths K., James M.N.G. J. Mol. Biol. 261:267-278(1996) [PubMed: 8757293] [Abstract] Cited for: X-RAY CRYSTALLOGRAPHY (2.2 ANGSTROMS). |
| + | Additional computationally mapped references. |
Cross-references
Sequence databases | |||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| M75154 mRNA. Translation: AAA59558.1. AC004799 Genomic DNA. No translation available. CH471139 Genomic DNA. Translation: EAW69591.1. BC096183 mRNA. Translation: AAH96183.1. BC096184 mRNA. Translation: AAH96184.1. BC096185 mRNA. Translation: AAH96185.1. BC096186 mRNA. Translation: AAH96186.1. M96628 Genomic DNA. Translation: AAB59364.1. M96839, M96838, M96837 Genomic DNA. Translation: AAB59493.1. M97911 Genomic DNA. No translation available. AF015449 AF015448 Genomic DNA. Translation: AAD21524.1. X56606 mRNA. Translation: CAA39943.1. X55668 mRNA. Translation: CAA39203.1. M29142 mRNA. Translation: AAA36342.1. Frameshift. X56132 mRNA. Translation: CAA39597.1. X56132 mRNA. Translation: CAA39598.1. Different initiation. | |||||||||||||
| IPI | IPI00027409. | ||||||||||||
| PIR | PRHU3. A45080. | ||||||||||||
| RefSeq | NP_002768.3. | ||||||||||||
| UniGene | Hs.928 | ||||||||||||
3D structure databases | |||||||||||||
| |||||||||||||
| ModBase | Search... | ||||||||||||
Protein-protein interaction databases | |||||||||||||
| IntAct | P24158. 1 interaction. | ||||||||||||
Protein family/group databases | |||||||||||||
| MEROPS | S01.134. | ||||||||||||
Proteomic databases | |||||||||||||
| PeptideAtlas | P24158. | ||||||||||||
| PRIDE | P24158. | ||||||||||||
Genome annotation databases | |||||||||||||
| Ensembl | ENSG00000196415. Homo sapiens. [Contig view] | ||||||||||||
| GeneID | 5657. | ||||||||||||
| KEGG | hsa:5657. | ||||||||||||
Organism-specific databases | |||||||||||||
| GeneCards | GC19P000794. | ||||||||||||
| H-InvDB | HIX0040155. | ||||||||||||
| HGNC | HGNC:9495. PRTN3. | ||||||||||||
| HPA | CAB017558. HPA005938. | ||||||||||||
| MIM | 177020. gene. | ||||||||||||
| Orphanet | 900. Wegener granulomatosis. | ||||||||||||
| PharmGKB | PA33842. | ||||||||||||
| GenAtlas | Search... | ||||||||||||
Phylogenomic databases | |||||||||||||
| HOGENOM | P24158. | ||||||||||||
| HOVERGEN | P24158. | ||||||||||||
| OMA | P24158. QGTQCLA. | ||||||||||||
Enzyme and pathway databases | |||||||||||||
| BRENDA | 3.4.21.76. 247. | ||||||||||||
Gene expression databases | |||||||||||||
| ArrayExpress | P24158. | ||||||||||||
| Bgee | P24158. | ||||||||||||
| CleanEx | HS_PRTN3. | ||||||||||||
| GermOnline | ENSG00000196415. Homo sapiens. | ||||||||||||
Family and domain databases | |||||||||||||
| InterPro | IPR018114. Peptidase_S1/S6_AS. IPR001254. Peptidase_S1_S6. IPR001314. Peptidase_S1A. [Graphical view] | ||||||||||||
| Pfam | PF00089. Trypsin. 1 hit. [Graphical view] | ||||||||||||
| PRINTS | PR00722. CHYMOTRYPSIN. | ||||||||||||
| SMART | SM00020. Tryp_SPc. 1 hit. [Graphical view] | ||||||||||||
| PROSITE | PS50240. TRYPSIN_DOM. 1 hit. PS00134. TRYPSIN_HIS. 1 hit. PS00135. TRYPSIN_SER. 1 hit. [Graphical view] | ||||||||||||
| ProtoNet | Search... | ||||||||||||
Other Resources | |||||||||||||
| NextBio | 21988. | ||||||||||||
| SOURCE | Search... | ||||||||||||
Entry information
| Entry name | PRTN3_HUMAN | ||||||||
| Accession | Primary (citable) accession number: P24158 Secondary accession number(s): P15637 Q9UQD8 | ||||||||
| Entry history |
| ||||||||
| Entry status | Reviewed (UniProtKB/Swiss-Prot) | ||||||||
| Annotation project | HPI (Human Proteome Initiative) | ||||||||
Relevant documents
| Human chromosome 19 Human chromosome 19: entries, gene names and cross-references to MIM |
| Human entries with polymorphisms or disease mutations List of human entries with polymorphisms or disease mutations |
| Human polymorphisms and disease mutations Index of human polymorphisms and disease mutations |
| MIM cross-references Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot |
| PDB cross-references Index of Protein Data Bank (PDB) cross-references |
| Peptidase families Classification of peptidase families and list of entries |
| SIMILARITY comments Index of protein domains and families |

Clusters with


