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Reviewed, UniProtKB/Swiss-Prot P98073 (ENTK_HUMAN)

Last modified January 19, 2010. Version 112. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (6) | Third-party data | Customize display text xml rdf/xml gff fasta
Names and origin · Protein attributes · General annotation (Comments) · Ontologies · Sequence annotation (Features) · Sequences · References · Cross-references · Entry information · Relevant documents

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Names and origin

Protein namesRecommended name:
    Enteropeptidase
    EC=3.4.21.9
Alternative name(s):
    Enterokinase
    Serine protease 7
Cleaved into the following 2 chains:
    1- Recommended name:
            Enteropeptidase non-catalytic heavy chain
    2- Recommended name:
            Enteropeptidase catalytic light chain
Gene names
Name: PRSS7
Synonyms: ENTK
OrganismHomo sapiens (Human) [Complete proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo

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Protein attributes

Sequence length1019 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at transcript level.

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General annotation (Comments)

Function

Responsible for initiating activation of pancreatic proteolytic proenzymes (trypsin, chymotrypsin and carboxypeptidase A). It catalyzes the conversion of trypsinogen to trypsin which in turn activates other proenzymes including chymotrypsinogen, procarboxypeptidases, and proelastases.

Catalytic activity

Activation of trypsinogen by selective cleavage of 6-Lys-|-Ile-7 bond.

Subunit structure

Heterodimer of a catalytic (light) chain and a multidomain (heavy) chain linked by a disulfide bond.

Subcellular location

Membrane; Single-pass type II membrane protein Probable.

Tissue specificity

Intestinal brush border.

Post-translational modification

The chains are derived from a single precursor that is cleaved by a trypsin-like protease.

Involvement in disease

Defects in PRSS7 are a cause of enterokinase deficiency [MIM:226200]; a life-threatening intestinal malabsorption disorder characterized by diarrhea and failure to thrive. Ref.2

Sequence similarities

Belongs to the peptidase S1 family.

Contains 2 CUB domains.

Contains 2 LDL-receptor class A domains.

Contains 1 MAM domain.

Contains 1 peptidase S1 domain.

Contains 1 SEA domain.

Contains 1 SRCR domain.

Sequence caution

The sequence CAB90389.1 differs from that shown. Reason: Erroneous gene model prediction.

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Ontologies

Keywords
   Cellular componentMembrane
   Coding sequence diversityPolymorphism
   DomainRepeat
Signal-anchor
Transmembrane
   Molecular functionHydrolase
Protease
Serine protease
   PTMDisulfide bond
Glycoprotein
Lipoprotein
Myristate
Zymogen
   Technical termComplete proteome
Gene Ontology (GO)
   Biological processproteolysis

Inferred from electronic annotation. Source: InterPro

   Cellular componentbrush border Ref.5

Traceable author statement. Source: ProtInc

integral to membrane

Inferred from electronic annotation. Source: UniProtKB-SubCell

   Molecular functionscavenger receptor activity

Inferred from electronic annotation. Source: InterPro

serine-type endopeptidase activity

Inferred from electronic annotation. Source: InterPro

Complete GO annotation...

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Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 784784Enteropeptidase non-catalytic heavy chain
PRO_0000027719
Chain785 – 1019235Enteropeptidase catalytic light chain
PRO_0000027720

Regions

Topological domain1 – 1818Cytoplasmic Potential
Transmembrane19 – 4729Signal-anchor for type II membrane protein Potential
Topological domain48 – 1019972Extracellular Potential
Domain52 – 169118SEA
Domain182 – 22342LDL-receptor class A 1
Domain225 – 334110CUB 1
Domain342 – 504163MAM
Domain524 – 634111CUB 2
Domain641 – 67939LDL-receptor class A 2
Domain678 – 77194SRCR
Domain785 – 1019235Peptidase S1

Sites

Active site8251Charge relay system By similarity
Active site8761Charge relay system By similarity
Active site9711Charge relay system By similarity

Amino acid modifications

Lipidation21N-myristoyl glycine Potential
Glycosylation1161N-linked (GlcNAc...) Potential
Glycosylation1471N-linked (GlcNAc...) Potential
Glycosylation1791N-linked (GlcNAc...) Potential
Glycosylation3281N-linked (GlcNAc...) Potential
Glycosylation3351N-linked (GlcNAc...) Potential
Glycosylation3881N-linked (GlcNAc...) Potential
Glycosylation4401N-linked (GlcNAc...) Potential
Glycosylation4701N-linked (GlcNAc...) Potential
Glycosylation5031N-linked (GlcNAc...) Potential
Glycosylation5341N-linked (GlcNAc...) Potential
Glycosylation6301N-linked (GlcNAc...) Potential
Glycosylation6821N-linked (GlcNAc...) Potential
Glycosylation7061N-linked (GlcNAc...) Potential
Glycosylation7251N-linked (GlcNAc...) Potential
Glycosylation8481N-linked (GlcNAc...) Potential
Glycosylation8871N-linked (GlcNAc...) Potential
Glycosylation9091N-linked (GlcNAc...) Potential
Glycosylation9491N-linked (GlcNAc...) Potential
Disulfide bond184 ↔ 197 By similarity
Disulfide bond191 ↔ 210 By similarity
Disulfide bond204 ↔ 221 By similarity
Disulfide bond225 ↔ 253 By similarity
Disulfide bond524 ↔ 552 By similarity
Disulfide bond643 ↔ 655 By similarity
Disulfide bond650 ↔ 668 By similarity
Disulfide bond662 ↔ 677 By similarity
Disulfide bond757 ↔ 767 By similarity
Disulfide bond772 ↔ 896Interchain (between heavy and light chains) By similarity
Disulfide bond810 ↔ 826 By similarity
Disulfide bond910 ↔ 977 By similarity
Disulfide bond941 ↔ 956 By similarity
Disulfide bond967 ↔ 995 By similarity

Natural variations

Natural variant651T → I: dbSNP rs35987974.
VAR_031686
Natural variant771K → R: dbSNP rs2824804.
VAR_021940
Natural variant1341E → Q: dbSNP rs2824790. Ref.2 Ref.1 Ref.4
VAR_031687
Natural variant5451S → C: dbSNP rs8134187.
VAR_031688
Natural variant6411E → K: dbSNP rs2273204.
VAR_020175
Natural variant6601N → H: dbSNP rs11088674.
VAR_024292
Natural variant7321S → P: dbSNP rs2824721. Ref.3
VAR_031689
Natural variant8281Y → C: dbSNP rs8130110.
VAR_031690

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Sequences

Sequence LengthMass (Da)Tools
P98073-1 [UniParc].

Last modified September 22, 2009. Version 2.
Checksum: F36AEB9738050352

FASTA1,019112,925
        10         20         30         40         50         60 
MGSKRGISSR HHSLSSYEIM FAALFAILVV LCAGLIAVSC LTIKESQRGA ALGQSHEARA 

        70         80         90        100        110        120 
TFKITSGVTY NPNLQDKLSV DFKVLAFDLQ QMIDEIFLSS NLKNEYKNSR VLQFENGSII 

       130        140        150        160        170        180 
VVFDLFFAQW VSDENVKEEL IQGLEANKSS QLVTFHIDLN SVDILDKLTT TSHLATPGNV 

       190        200        210        220        230        240 
SIECLPGSSP CTDALTCIKA DLFCDGEVNC PDGSDEDNKM CATVCDGRFL LTGSSGSFQA 

       250        260        270        280        290        300 
THYPKPSETS VVCQWIIRVN QGLSIKLSFD DFNTYYTDIL DIYEGVGSSK ILRASIWETN 

       310        320        330        340        350        360 
PGTIRIFSNQ VTATFLIESD ESDYVGFNAT YTAFNSSELN NYEKINCNFE DGFCFWVQDL 

       370        380        390        400        410        420 
NDDNEWERIQ GSTFSPFTGP NFDHTFGNAS GFYISTPTGP GGRQERVGLL SLPLDPTLEP 

       430        440        450        460        470        480 
ACLSFWYHMY GENVHKLSIN ISNDQNMEKT VFQKEGNYGD NWNYGQVTLN ETVKFKVAFN 

       490        500        510        520        530        540 
AFKNKILSDI ALDDISLTYG ICNGSLYPEP TLVPTPPPEL PTDCGGPFEL WEPNTTFSST 

       550        560        570        580        590        600 
NFPNSYPNLA FCVWILNAQK GKNIQLHFQE FDLENINDVV EIRDGEEADS LLLAVYTGPG 

       610        620        630        640        650        660 
PVKDVFSTTN RMTVLLITND VLARGGFKAN FTTGYHLGIP EPCKADHFQC KNGECVPLVN 

       670        680        690        700        710        720 
LCDGHLHCED GSDEADCVRF FNGTTNNNGL VRFRIQSIWH TACAENWTTQ ISNDVCQLLG 

       730        740        750        760        770        780 
LGSGNSSKPI FSTDGGPFVK LNTAPDGHLI LTPSQQCLQD SLIRLQCNHK SCGKKLAAQD 

       790        800        810        820        830        840 
ITPKIVGGSN AKEGAWPWVV GLYYGGRLLC GASLVSSDWL VSAAHCVYGR NLEPSKWTAI 

       850        860        870        880        890        900 
LGLHMKSNLT SPQTVPRLID EIVINPHYNR RRKDNDIAMM HLEFKVNYTD YIQPICLPEE 

       910        920        930        940        950        960 
NQVFPPGRNC SIAGWGTVVY QGTTANILQE ADVPLLSNER CQQQMPEYNI TENMICAGYE 

       970        980        990       1000       1010 
EGGIDSCQGD SGGPLMCQEN NRWFLAGVTS FGYKCALPNR PGVYARVSRF TEWIQSFLH

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References

« Hide 'large scale' references
[1]"cDNA sequence and chromosomal localization of human enterokinase, the proteolytic activator of trypsinogen."
Kitamoto Y., Veile R.A., Donis-Keller H., Sadler J.E.
Biochemistry 34:4562-4568(1995) [PubMed: 7718557] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA], VARIANT GLN-134.
Tissue: Duodenum.
[2]"Mutations in the proenteropeptidase gene are the molecular cause of congenital enteropeptidase deficiency."
Holzinger A., Maier E.M., Buck C., Mayerhofer P.U., Kappler M., Haworth J.C., Moroz S.P., Hadorn H.-B., Sadler J.E., Roscher A.A.
Am. J. Hum. Genet. 70:20-25(2002) [PubMed: 11719902] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], VARIANT GLN-134, INVOLVEMENT IN ENTEROKINASE DEFICIENCY.
[3]"The DNA sequence of human chromosome 21."
Hattori M., Fujiyama A., Taylor T.D., Watanabe H., Yada T., Park H.-S., Toyoda A., Ishii K., Totoki Y., Choi D.-K., Groner Y., Soeda E., Ohki M., Takagi T., Sakaki Y., Taudien S., Blechschmidt K., Polley A. expand/collapse author list , Menzel U., Delabar J., Kumpf K., Lehmann R., Patterson D., Reichwald K., Rump A., Schillhabel M., Schudy A., Zimmermann W., Rosenthal A., Kudoh J., Shibuya K., Kawasaki K., Asakawa S., Shintani A., Sasaki T., Nagamine K., Mitsuyama S., Antonarakis S.E., Minoshima S., Shimizu N., Nordsiek G., Hornischer K., Brandt P., Scharfe M., Schoen O., Desario A., Reichelt J., Kauer G., Bloecker H., Ramser J., Beck A., Klages S., Hennig S., Riesselmann L., Dagand E., Wehrmeyer S., Borzym K., Gardiner K., Nizetic D., Francis F., Lehrach H., Reinhardt R., Yaspo M.-L.
Nature 405:311-319(2000) [PubMed: 10830953] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA], VARIANT PRO-732.
[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 GLN-134.
[5]"Enterokinase, the initiator of intestinal digestion, is a mosaic protease composed of a distinctive assortment of domains."
Kitamoto Y., Yuan X., Wu Q., McCourt D.W., Sadler J.E.
Proc. Natl. Acad. Sci. U.S.A. 91:7588-7592(1994) [PubMed: 8052624] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 749-1019.
Tissue: Duodenum.
+Additional computationally mapped references.

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Cross-references

Sequence databases

EMBL
GenBank
DDBJ		U09860 mRNA. Translation: AAC50138.1.
Y19124 expand/collapse EMBL AC list , Y19125, Y19126, Y19127, Y19128, Y19129, Y19130, Y19131, Y19132, Y19133, Y19134, Y19135, Y19136, Y19137, Y19138, Y19139, Y19140, Y19141, Y19142, Y19143 Genomic DNA. Translation: CAB65555.1.
AL163217 Genomic DNA. Translation: CAB90389.1. Sequence problems.
AL163218 Genomic DNA. Translation: CAB90392.1.
BC111749 mRNA. Translation: AAI11750.1.
IPIIPI00023788.
PIRA56318.
RefSeqNP_002763.2.
UniGeneHs.149473

3D structure databases

ModBaseSearch...

Protein-protein interaction databases

STRINGP98073.

Protein family/group databases

MEROPSS01.156.

Genome annotation databases

EnsemblENST00000284885; ENSP00000284885; ENSG00000154646; Homo sapiens. [Genome view]
GeneID5651.
KEGGhsa:5651.
UCSCuc002ykw.1. human.

Organism-specific databases

CTD5651.
GeneCardsGC21M018563.
H-InvDBHIX0040924.
HGNCHGNC:9490. PRSS7.
HPAHPA015611.
MIM226200. phenotype.
606635. gene.
Orphanet168601. Congenital enteropathy due to enteropeptidase deficiency.
PharmGKBPA33839.
GenAtlasSearch...

Phylogenomic databases

eggNOGprNOG08056.
HOGENOMHBG445315.
HOVERGENP98073.
InParanoidP98073.

Enzyme and pathway databases

BRENDA3.4.21.9. 247.

Gene expression databases

ArrayExpressP98073.
BgeeP98073.
CleanExHS_PRSS7.
GenevestigatorP98073.
GermOnlineENSG00000154646. Homo sapiens.

Family and domain databases

InterProIPR000859. CUB.
IPR002172. LDL_rcpt_classA_cys-rich_rpt.
IPR000998. MAM.
IPR018114. Peptidase_S1/S6_AS.
IPR001254. Peptidase_S1_S6.
IPR001314. Peptidase_S1A.
IPR000082. SEA.
IPR009003. Ser/Cys_Pept_Trypsin-like.
IPR001190. Srcr_rcpt.
IPR017448. Srcr_rcpt-rel.
[Graphical view]
Gene3DG3DSA:2.60.120.290. CUB. 2 hits.
G3DSA:4.10.400.10. LDL_rcpt_classA_cys-rich. 1 hit.
PfamPF00431. CUB. 2 hits.
PF00057. Ldl_recept_a. 2 hits.
PF00629. MAM. 1 hit.
PF01390. SEA. 1 hit.
PF00530. SRCR. 1 hit.
PF00089. Trypsin. 1 hit.
[Graphical view]
PRINTSPR00722. CHYMOTRYPSIN.
PR00261. LDLRECEPTOR.
SMARTSM00042. CUB. 2 hits.
SM00192. LDLa. 2 hits.
SM00137. MAM. 1 hit.
SM00200. SEA. 1 hit.
SM00202. SR. 1 hit.
SM00020. Tryp_SPc. 1 hit.
[Graphical view]
PROSITEPS01180. CUB. 2 hits.
PS01209. LDLRA_1. 2 hits.
PS50068. LDLRA_2. 2 hits.
PS00740. MAM_1. 1 hit.
PS50060. MAM_2. 1 hit.
PS50024. SEA. 1 hit.
PS00420. SRCR_1. False negative.
PS50287. SRCR_2. 1 hit.
PS50240. TRYPSIN_DOM. 1 hit.
PS00134. TRYPSIN_HIS. 1 hit.
PS00135. TRYPSIN_SER. 1 hit.
[Graphical view]
ProtoNetSearch...

Other Resources

NextBio21958.
SOURCESearch...

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Entry information

Entry nameENTK_HUMAN
AccessionPrimary (citable) accession number: P98073
Secondary accession number(s): Q2NKL7
Entry history
Integrated into UniProtKB/Swiss-Prot: February 1, 1996
Last sequence update: September 22, 2009
Last modified: January 19, 2010
This is version 112 of the entry and version 2 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation projectHPI (Human Proteome Initiative)
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.

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Relevant documents

Human chromosome 21

Human chromosome 21: 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

Peptidase families

Classification of peptidase families and list of entries

SIMILARITY comments

Index of protein domains and families

Names and origin · Protein attributes · General annotation (Comments) · Ontologies · Sequence annotation (Features) · Sequences · References · Cross-references · Entry information · Relevant documents