Skip Header

You are using a version of Internet Explorer that may not display all features of this website. Please upgrade to a modern browser.
Contribute Send feedback
Read comments (?) or add your own

O14841 (OPLA_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified May 14, 2014. Version 108. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (5) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
5-oxoprolinase

EC=3.5.2.9
Alternative name(s):
5-oxo-L-prolinase
Short name=5-OPase
Pyroglutamase
Gene names
Name:OPLAH
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length1288 AA.
Sequence statusComplete.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

Catalyzes the cleavage of 5-oxo-L-proline to form L-glutamate coupled to the hydrolysis of ATP to ADP and inorganic phosphate.

Catalytic activity

ATP + 5-oxo-L-proline + 2 H2O = ADP + phosphate + L-glutamate.

Subunit structure

Homodimer By similarity.

Involvement in disease

5-oxoprolinase deficiency (OPLAHD) [MIM:260005]: A disorder characterized by calcium oxalate/carbonate urolithiasis, and excessive urinary 5-oxo-L-proline. Affected individuals have recurrent episodes of vomiting, diarrhea, and abdominal pain.
Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.6

Sequence similarities

Belongs to the oxoprolinase family.

Sequence caution

The sequence AAB81519.1 differs from that shown. Reason: Frameshift at positions 1046, 1068 and 1080.

The sequence CAB46426.2 differs from that shown. Reason: Frameshift at several positions.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 128812885-oxoprolinase
PRO_0000208577

Natural variations

Natural variant2841S → R.
Corresponds to variant rs3935209 [ dbSNP | Ensembl ].
VAR_050425

Experimental info

Sequence conflict1084 – 10896QRVVDV → NAWWMF in AAB81519. Ref.4

Sequences

Sequence LengthMass (Da)Tools
O14841 [UniParc].

Last modified February 15, 2005. Version 3.
Checksum: 999AC0FA497750F2

FASTA1,288137,457
        10         20         30         40         50         60 
MGSPEGRFHF AIDRGGTFTD VFAQCPGGHV RVLKLLSEDP ANYADAPTEG IRRILEQEAG 

        70         80         90        100        110        120 
MLLPRDQPLD SSHIASIRMG TTVATNALLE RKGERVALLV TRGFRDLLHI GTQARGDLFD 

       130        140        150        160        170        180 
LAVPMPEVLY EEVLEVDERV VLHRGEAGTG TPVKGRTGDL LEVQQPVDLG ALRGKLEGLL 

       190        200        210        220        230        240 
SRGIRSLAVV LMHSYTWAQH EQQVGVLARE LGFTHVSLSS EAMPMVRIVP RGHTACADAY 

       250        260        270        280        290        300 
LTPAIQRYVQ GFCRGFQGQL KDVQVLFMRS DGGLAPMDTF SGSSAVLSGP AGGVVGYSAT 

       310        320        330        340        350        360 
TYQQEGGQPV IGFDMGGTST DVSRYAGEFE HVFEASTAGV TLQAPQLDIN TVAAGGGSRL 

       370        380        390        400        410        420 
FFRSGLFVVG PESAGAHPGP ACYRKGGPVT VTDANLVLGR LLPASFPCIF GPGENQPLSP 

       430        440        450        460        470        480 
EASRKALEAV ATEVNSFLTN GPCPASPLSL EEVAMGFVRV ANEAMCRPIR ALTQARGHDP 

       490        500        510        520        530        540 
SAHVLACFGG AGGQHACAIA RALGMDTVHI HRHSGLLSAL GLALADVVHE AQEPCSLLYA 

       550        560        570        580        590        600 
PETFVQLDQR LSRLEEQCVD ALQAQGFPRS QISTESFLHL RYQGTDCALM VSAHQHPATA 

       610        620        630        640        650        660 
RSPRAGDFGA AFVERYMREF GFVIPERPVV VDDVRVRGTG RSGLRLEDAP KAQTGPPRVD 

       670        680        690        700        710        720 
KMTQCYFEGG YQETPVYLLA ELGYGHKLHG PCLIIDSNST ILVEPGCQAE VTKTGDICIS 

       730        740        750        760        770        780 
VGAEVPGTVG PQLDPIQLSI FSHRFMSIAE QMGRILQRTA ISTNIKERLD FSCALFGPDG 

       790        800        810        820        830        840 
GLVSNAPHIP VHLGAMQETV QFQIQHLGAD LHPGDVLLSN HPSAGGSHLP DLTVITPVFW 

       850        860        870        880        890        900 
PGQTRPVFYV ASRGHHADIG GITPGSMPPH STMLQQEGAV FLSFKLVQGG VFQEEAVTEA 

       910        920        930        940        950        960 
LRAPGKVPNC SGTRNLHDNL SDLRAQVAAN QKGIQLVGEL IGQYGLDVVQ AYMGHIQANA 

       970        980        990       1000       1010       1020 
ELAVRDMLRA FGTSRQARGL PLEVSSEDHM DDGSPIRLRV QISLSQGSAV FDFSGTGPEV 

      1030       1040       1050       1060       1070       1080 
FGNLNAPRAV TLSALIYCLR CLVGRDIPLN QGCLAPVRVV IPRGSILDPS PEAAVVGGNV 

      1090       1100       1110       1120       1130       1140 
LTSQRVVDVI LGAFGACAAS QGCMNNVTLG NAHMGYYETV AGGAGAGPSW HGRSGVHSHM 

      1150       1160       1170       1180       1190       1200 
TNTRITDPEI LESRYPVILR RFELRRGSGG RGRFRGGDGV TRELLFREEA LLSVLTERRA 

      1210       1220       1230       1240       1250       1260 
FRPYGLHGGE PGARGLNLLI RKNGRTVNLG GKTSVTVYPG DVFCLHTPGG GGYGDPEDPA 

      1270       1280 
PPPGSPPQAL AFPEHGSVYE YRRAQEAV 

« Hide

References

« Hide 'large scale' references
[1]"Bovine 5-oxo-L-prolinase: simple assay method, purification, cDNA cloning, and detection of mRNA in the coronary artery."
Watanabe T., Abe K., Ishikawa H., Iijima Y.
Biol. Pharm. Bull. 27:288-294(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA].
[2]"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].
[3]"The full-ORF clone resource of the German cDNA consortium."
Bechtel S., Rosenfelder H., Duda A., Schmidt C.P., Ernst U., Wellenreuther R., Mehrle A., Schuster C., Bahr A., Bloecker H., Heubner D., Hoerlein A., Michel G., Wedler H., Koehrer K., Ottenwaelder B., Poustka A., Wiemann S., Schupp I.
BMC Genomics 8:399-399(2007) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 229-1288.
Tissue: Testis.
[4]"Human 5-oxo-L-prolinase partial sequence."
Debella L.R., Wood S.
Submitted (SEP-1997) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 901-1096.
[5]"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].
[6]"5-Oxoprolinase deficiency: report of the first human OPLAH mutation."
Almaghlouth I., Mohamed J., Al-Amoudi M., Al-Ahaidib L., Al-Odaib A., Alkuraya F.
Clin. Genet. 82:193-196(2012) [PubMed] [Europe PMC] [Abstract]
Cited for: INVOLVEMENT IN OPLAHD.
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
AB122018 mRNA. Translation: BAD13434.1.
BC142672 mRNA. Translation: AAI42673.1.
AL096750 mRNA. Translation: CAB46426.2. Frameshift.
AH005594 Genomic DNA. Translation: AAB81519.1. Frameshift.
PIRT12537.
RefSeqNP_060040.1. NM_017570.4.
UniGeneHs.305882.

3D structure databases

ProteinModelPortalO14841.
SMRO14841. Positions 450-525.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid117922. 1 interaction.
IntActO14841. 1 interaction.
STRING9606.ENSP00000412071.

Chemistry

DrugBankDB00142. L-Glutamic Acid.

PTM databases

PhosphoSiteO14841.

Proteomic databases

MaxQBO14841.
PaxDbO14841.
PRIDEO14841.

Protocols and materials databases

DNASU26873.
StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000426825; ENSP00000475943; ENSG00000178814.
GeneID26873.
KEGGhsa:26873.
UCSCuc003zar.3. human.

Organism-specific databases

CTD26873.
GeneCardsGC08M145106.
HGNCHGNC:8149. OPLAH.
HPAHPA026562.
HPA028260.
MIM260005. phenotype.
614243. gene.
neXtProtNX_O14841.
Orphanet33572. 5-oxoprolinase deficiency.
PharmGKBPA31935.
GenAtlasSearch...

Phylogenomic databases

eggNOGCOG0146.
HOGENOMHOG000047200.
HOVERGENHBG007870.
InParanoidO14841.
KOK01469.
OMAVEAYMGH.
PhylomeDBO14841.

Enzyme and pathway databases

ReactomeREACT_111217. Metabolism.

Gene expression databases

CleanExHS_OPLAH.
GenevestigatorO14841.

Family and domain databases

InterProIPR008040. Hydant_A_N.
IPR002821. Hydantoinase_A.
IPR003692. Hydantoinase_B.
[Graphical view]
PfamPF05378. Hydant_A_N. 1 hit.
PF01968. Hydantoinase_A. 1 hit.
PF02538. Hydantoinase_B. 1 hit.
[Graphical view]
ProtoNetSearch...

Other

GenomeRNAi26873.
NextBio49396.
PROO14841.
SOURCESearch...

Entry information

Entry nameOPLA_HUMAN
AccessionPrimary (citable) accession number: O14841
Secondary accession number(s): A5PKY8, Q75W65, Q9Y4Q0
Entry history
Integrated into UniProtKB/Swiss-Prot: December 15, 1998
Last sequence update: February 15, 2005
Last modified: May 14, 2014
This is version 108 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

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 8

Human chromosome 8: entries, gene names and cross-references to MIM