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P24158 (PRTN3_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified July 9, 2014. Version 159. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (7) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Web links·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Myeloblastin

EC=3.4.21.76
Alternative name(s):
AGP7
C-ANCA antigen
Leukocyte proteinase 3
Short name=PR-3
Short name=PR3
Neutrophil proteinase 4
Short name=NP-4
P29
Wegener autoantigen
Gene names
Name:PRTN3
Synonyms:MBN
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length256 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence 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-. Ref.18

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.

The sequence CAA39598.1 differs from that shown. Reason: Erroneous initiation.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Signal peptide1 – 2525
Propeptide26 – 272
PRO_0000027707
Chain28 – 248221Myeloblastin
PRO_0000027708
Propeptide249 – 2568
PRO_0000027709

Regions

Domain28 – 248221Peptidase S1

Sites

Active site711Charge relay system
Active site1181Charge relay system
Active site2031Charge relay system

Amino acid modifications

Glycosylation1291N-linked (GlcNAc...) Potential
Glycosylation1741N-linked (GlcNAc...)
Disulfide bond56 ↔ 72
Disulfide bond152 ↔ 209
Disulfide bond182 ↔ 188
Disulfide bond199 ↔ 224

Natural variations

Natural variant1191V → I. Ref.1 Ref.3 Ref.4 Ref.7 Ref.10 Ref.11 Ref.20
Corresponds to variant rs351111 [ dbSNP | Ensembl ].
VAR_011691
Natural variant1351A → T. Ref.1 Ref.6 Ref.10 Ref.11
Corresponds to variant rs1042281 [ dbSNP | Ensembl ].
VAR_011713
Natural variant1361T → S. Ref.1 Ref.6 Ref.10 Ref.11
Corresponds to variant rs1042282 [ dbSNP | Ensembl ].
VAR_011714

Experimental info

Sequence conflict21A → R in CAA39203. Ref.9
Sequence conflict381S → I AA sequence Ref.19
Sequence conflict401P → PI AA sequence Ref.19
Sequence conflict461Q → E AA sequence Ref.13
Sequence conflict461Q → E AA sequence Ref.17
Sequence conflict481R → A AA sequence Ref.14
Sequence conflict641S → D AA sequence Ref.12
Sequence conflict701A → P in AAA59558. Ref.1
Sequence conflict2551Missing in CAA39203. Ref.9

Secondary structure

......................................... 256
Helix Strand Turn

Details...

Sequences

Sequence LengthMass (Da)Tools
P24158 [UniParc].

Last modified December 15, 1998. Version 3.
Checksum: CBECA36D8C4B2A40

FASTA25627,807
        10         20         30         40         50         60 
MAHRPPSPAL ASVLLALLLS GAARAAEIVG GHEAQPHSRP YMASLQMRGN PGSHFCGGTL 

        70         80         90        100        110        120 
IHPSFVLTAA HCLRDIPQRL VNVVLGAHNV RTQEPTQQHF SVAQVFLNNY DAENKLNDVL 

       130        140        150        160        170        180 
LIQLSSPANL SASVATVQLP QQDQPVPHGT QCLAMGWGRV GAHDPPAQVL QELNVTVVTF 

       190        200        210        220        230        240 
FCRPHNICTF VPRRKAGICF GDSGGPLICD GIIQGIDSFV IWGCATRLFP DFFTRVALYV 

       250 
DWIRSTLRRV EAKGRP 

« Hide

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] [Europe PMC] [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. expand/collapse author list , Caoile C., Chan Y.M., Christensen M., Cleland C.A., Copeland A., Dalin E., Dehal P., Denys M., Detter J.C., Escobar J., Flowers D., Fotopulos D., Garcia C., Georgescu A.M., Glavina T., Gomez M., Gonzales E., Groza M., Hammon N., Hawkins T., Haydu L., Ho I., Huang W., Israni S., Jett J., Kadner K., Kimball H., Kobayashi A., Larionov V., Leem S.-H., Lopez F., Lou Y., Lowry S., Malfatti S., Martinez D., McCready P.M., Medina C., Morgan J., Nelson K., Nolan M., Ovcharenko I., Pitluck S., Pollard M., Popkie A.P., Predki P., Quan G., Ramirez L., Rash S., Retterer J., Rodriguez A., Rogers S., Salamov A., Salazar A., She X., Smith D., Slezak T., Solovyev V., Thayer N., Tice H., Tsai M., Ustaszewska A., Vo N., Wagner M., Wheeler J., Wu K., Xie G., Yang J., Dubchak I., Furey T.S., DeJong P., Dickson M., Gordon D., Eichler E.E., Pennacchio L.A., Richardson P., Stubbs L., Rokhsar D.S., Myers R.M., Rubin E.M., Lucas S.M.
Nature 428:529-535(2004) [PubMed] [Europe PMC] [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. expand/collapse author list , Yooseph S., Lu F., Nusskern D.R., Shue B.C., Zheng X.H., Zhong F., Delcher A.L., Huson D.H., Kravitz S.A., Mouchard L., Reinert K., Remington K.A., Clark A.G., Waterman M.S., Eichler E.E., Adams M.D., Hunkapiller M.W., Myers E.W., 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] [Europe PMC] [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] [Europe PMC] [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] [Europe PMC] [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] [Europe PMC] [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] [Europe PMC] [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] [Europe PMC] [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] [Europe PMC] [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] [Europe PMC] [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] [Europe PMC] [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] [Europe PMC] [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] [Europe PMC] [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] [Europe PMC] [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] [Europe PMC] [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] [Europe PMC] [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] [Europe PMC] [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] [Europe PMC] [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] [Europe PMC] [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] [Europe PMC] [Abstract]
Cited for: IDENTITY OF WEGENER'S AUTOANTIGEN WITH PROTEINASE 3.
[22]"Initial characterization of the human central proteome."
Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J.
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
[23]"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] [Europe PMC] [Abstract]
Cited for: X-RAY CRYSTALLOGRAPHY (2.2 ANGSTROMS).
+Additional computationally mapped references.

Web resources

Wikipedia

Proteinase 3 entry

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
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.
AH005293 Genomic DNA. Translation: AAB59493.1.
M97911 Genomic DNA. No translation available.
AH007523 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.
CCDSCCDS32860.1.
PIRPRHU3. A45080.
RefSeqNP_002768.3. NM_002777.3.
UniGeneHs.928.

3D structure databases

PDBe
RCSB-PDB
PDBj
EntryMethodResolution (Å)ChainPositionsPDBsum
1FUJX-ray2.20A/B/C/D28-248[»]
ProteinModelPortalP24158.
SMRP24158. Positions 28-248.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid111638. 6 interactions.
IntActP24158. 2 interactions.
MINTMINT-4054660.

Chemistry

BindingDBP24158.
ChEMBLCHEMBL3900.

Protein family/group databases

MEROPSS01.134.

Polymorphism databases

DMDM6174926.

Proteomic databases

PaxDbP24158.
PeptideAtlasP24158.
PRIDEP24158.

Protocols and materials databases

DNASU5657.
StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000234347; ENSP00000234347; ENSG00000196415.
GeneID5657.
KEGGhsa:5657.
UCSCuc002lqa.1. human.

Organism-specific databases

CTD5657.
GeneCardsGC19P000840.
HGNCHGNC:9495. PRTN3.
HPACAB017558.
HPA005938.
MIM177020. gene.
neXtProtNX_P24158.
Orphanet900. Granulomatosis with polyangiitis.
PharmGKBPA33842.
GenAtlasSearch...

Phylogenomic databases

eggNOGCOG5640.
HOVERGENHBG013304.
InParanoidP24158.
KOK01350.
OMAHFSVAQV.
OrthoDBEOG7MKW6Q.
PhylomeDBP24158.
TreeFamTF335284.

Enzyme and pathway databases

BRENDA3.4.21.76. 2681.

Gene expression databases

BgeeP24158.
CleanExHS_PRTN3.
GenevestigatorP24158.

Family and domain databases

InterProIPR001254. Peptidase_S1.
IPR018114. Peptidase_S1_AS.
IPR001314. Peptidase_S1A.
IPR009003. Trypsin-like_Pept_dom.
[Graphical view]
PfamPF00089. Trypsin. 1 hit.
[Graphical view]
PRINTSPR00722. CHYMOTRYPSIN.
SMARTSM00020. Tryp_SPc. 1 hit.
[Graphical view]
SUPFAMSSF50494. SSF50494. 1 hit.
PROSITEPS50240. TRYPSIN_DOM. 1 hit.
PS00134. TRYPSIN_HIS. 1 hit.
PS00135. TRYPSIN_SER. 1 hit.
[Graphical view]
ProtoNetSearch...

Other

EvolutionaryTraceP24158.
GeneWikiProteinase_3.
GenomeRNAi5657.
NextBio21988.
PROP24158.
SOURCESearch...

Entry information

Entry namePRTN3_HUMAN
AccessionPrimary (citable) accession number: P24158
Secondary accession number(s): P15637 expand/collapse secondary AC list , P18078, Q4VB08, Q4VB09, Q6LBM7, Q6LBN2, Q9UD25, Q9UQD8
Entry history
Integrated into UniProtKB/Swiss-Prot: March 1, 1992
Last sequence update: December 15, 1998
Last modified: July 9, 2014
This is version 159 of the entry and version 3 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Relevant documents

SIMILARITY comments

Index of protein domains and families

Peptidase families

Classification of peptidase families and list of entries

PDB cross-references

Index of Protein Data Bank (PDB) cross-references

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

Human polymorphisms and disease mutations

Index of human polymorphisms and disease mutations

Human entries with polymorphisms or disease mutations

List of human entries with polymorphisms or disease mutations

Human chromosome 19

Human chromosome 19: entries, gene names and cross-references to MIM