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UniProtKB - O14841 (OPLA_HUMAN)
Protein
5-oxoprolinase
Gene
OPLAH
Organism
Homo sapiens (Human)
Status
Functioni
Catalyzes the cleavage of 5-oxo-L-proline to form L-glutamate coupled to the hydrolysis of ATP to ADP and inorganic phosphate.
Catalytic activityi
- EC:3.5.2.9
GO - Molecular functioni
- 5-oxoprolinase (ATP-hydrolyzing) activity Source: GO_Central
- ATP binding Source: UniProtKB-KW
- identical protein binding Source: IntAct
GO - Biological processi
- glutathione metabolic process Source: GO_Central
Keywordsi
Molecular function | Hydrolase |
Ligand | ATP-binding, Nucleotide-binding |
Enzyme and pathway databases
BRENDAi | 3.5.2.9, 2681 |
PathwayCommonsi | O14841 |
Reactomei | R-HSA-174403, Glutathione synthesis and recycling R-HSA-5578998, Defective OPLAH causes OPLAHD |
SignaLinki | O14841 |
Names & Taxonomyi
Protein namesi | Recommended name: 5-oxoprolinase (EC:3.5.2.9)Alternative name(s): 5-oxo-L-prolinase Short name: 5-OPase Pyroglutamase |
Gene namesi | Name:OPLAH |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:8149, OPLAH |
MIMi | 614243, gene |
neXtProti | NX_O14841 |
VEuPathDBi | HostDB:ENSG00000178814 |
Pathology & Biotechi
Involvement in diseasei
5-oxoprolinase deficiency (OPLAHD)1 Publication
The disease is caused by variants affecting the gene represented in this entry.
Disease descriptionA 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.
Related information in OMIMOrganism-specific databases
DisGeNETi | 26873 |
MalaCardsi | OPLAH |
MIMi | 260005, phenotype |
OpenTargetsi | ENSG00000178814 |
Orphaneti | 33572, 5-oxoprolinase deficiency |
PharmGKBi | PA31935 |
Miscellaneous databases
Pharosi | O14841, Tbio |
Chemistry databases
DrugBanki | DB00142, Glutamic acid |
Genetic variation databases
BioMutai | OPLAH |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
ChainiPRO_0000208577 | 1 – 1288 | 5-oxoprolinaseAdd BLAST | 1288 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 151 | PhosphothreonineCombined sources | 1 | |
Modified residuei | 1265 | PhosphoserineBy similarity | 1 |
Keywords - PTMi
PhosphoproteinProteomic databases
EPDi | O14841 |
jPOSTi | O14841 |
MassIVEi | O14841 |
MaxQBi | O14841 |
PeptideAtlasi | O14841 |
PRIDEi | O14841 |
ProteomicsDBi | 48272 |
PTM databases
GlyGeni | O14841, 1 site, 1 O-linked glycan (1 site) |
iPTMneti | O14841 |
MetOSitei | O14841 |
PhosphoSitePlusi | O14841 |
Expressioni
Gene expression databases
Bgeei | ENSG00000178814, Expressed in heart left ventricle and 174 other tissues |
ExpressionAtlasi | O14841, baseline and differential |
Genevisiblei | O14841, HS |
Organism-specific databases
HPAi | ENSG00000178814, Tissue enhanced (testis) |
Interactioni
Subunit structurei
Homodimer.
By similarityBinary interactionsi
O14841
With | #Exp. | IntAct |
---|---|---|
itself | 3 | EBI-3938544,EBI-3938544 |
POU6F2 [P78424] | 3 | EBI-3938544,EBI-12029004 |
TPK1 [Q9H3S4] | 3 | EBI-3938544,EBI-7054500 |
GO - Molecular functioni
- identical protein binding Source: IntAct
Protein-protein interaction databases
BioGRIDi | 117922, 10 interactors |
IntActi | O14841, 5 interactors |
MINTi | O14841 |
STRINGi | 9606.ENSP00000480476 |
Miscellaneous databases
RNActi | O14841, protein |
Family & Domainsi
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 1248 – 1272 | DisorderedSequence analysisAdd BLAST | 25 |
Compositional bias
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Compositional biasi | 1255 – 1269 | Pro residuesSequence analysisAdd BLAST | 15 |
Sequence similaritiesi
Belongs to the oxoprolinase family.Curated
Phylogenomic databases
eggNOGi | KOG1939, Eukaryota |
GeneTreei | ENSGT00390000013463 |
HOGENOMi | CLU_002157_0_0_1 |
InParanoidi | O14841 |
OMAi | MVVTKGF |
OrthoDBi | 62698at2759 |
PhylomeDBi | O14841 |
Family and domain databases
InterProi | View protein in InterPro IPR008040, Hydant_A_N IPR002821, Hydantoinase_A IPR003692, Hydantoinase_B IPR045079, Oxoprolinase_fam |
PANTHERi | PTHR11365, PTHR11365, 1 hit |
Pfami | View protein in Pfam PF05378, Hydant_A_N, 1 hit PF01968, Hydantoinase_A, 1 hit PF02538, Hydantoinase_B, 1 hit |
(1+)i Sequence
Sequence statusi: Complete.
This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All
O14841-1 [UniParc]FASTAAdd to basket
10 20 30 40 50
MGSPEGRFHF AIDRGGTFTD VFAQCPGGHV RVLKLLSEDP ANYADAPTEG
60 70 80 90 100
IRRILEQEAG MLLPRDQPLD SSHIASIRMG TTVATNALLE RKGERVALLV
110 120 130 140 150
TRGFRDLLHI GTQARGDLFD LAVPMPEVLY EEVLEVDERV VLHRGEAGTG
160 170 180 190 200
TPVKGRTGDL LEVQQPVDLG ALRGKLEGLL SRGIRSLAVV LMHSYTWAQH
210 220 230 240 250
EQQVGVLARE LGFTHVSLSS EAMPMVRIVP RGHTACADAY LTPAIQRYVQ
260 270 280 290 300
GFCRGFQGQL KDVQVLFMRS DGGLAPMDTF SGSSAVLSGP AGGVVGYSAT
310 320 330 340 350
TYQQEGGQPV IGFDMGGTST DVSRYAGEFE HVFEASTAGV TLQAPQLDIN
360 370 380 390 400
TVAAGGGSRL FFRSGLFVVG PESAGAHPGP ACYRKGGPVT VTDANLVLGR
410 420 430 440 450
LLPASFPCIF GPGENQPLSP EASRKALEAV ATEVNSFLTN GPCPASPLSL
460 470 480 490 500
EEVAMGFVRV ANEAMCRPIR ALTQARGHDP SAHVLACFGG AGGQHACAIA
510 520 530 540 550
RALGMDTVHI HRHSGLLSAL GLALADVVHE AQEPCSLLYA PETFVQLDQR
560 570 580 590 600
LSRLEEQCVD ALQAQGFPRS QISTESFLHL RYQGTDCALM VSAHQHPATA
610 620 630 640 650
RSPRAGDFGA AFVERYMREF GFVIPERPVV VDDVRVRGTG RSGLRLEDAP
660 670 680 690 700
KAQTGPPRVD KMTQCYFEGG YQETPVYLLA ELGYGHKLHG PCLIIDSNST
710 720 730 740 750
ILVEPGCQAE VTKTGDICIS VGAEVPGTVG PQLDPIQLSI FSHRFMSIAE
760 770 780 790 800
QMGRILQRTA ISTNIKERLD FSCALFGPDG GLVSNAPHIP VHLGAMQETV
810 820 830 840 850
QFQIQHLGAD LHPGDVLLSN HPSAGGSHLP DLTVITPVFW PGQTRPVFYV
860 870 880 890 900
ASRGHHADIG GITPGSMPPH STMLQQEGAV FLSFKLVQGG VFQEEAVTEA
910 920 930 940 950
LRAPGKVPNC SGTRNLHDNL SDLRAQVAAN QKGIQLVGEL IGQYGLDVVQ
960 970 980 990 1000
AYMGHIQANA ELAVRDMLRA FGTSRQARGL PLEVSSEDHM DDGSPIRLRV
1010 1020 1030 1040 1050
QISLSQGSAV FDFSGTGPEV FGNLNAPRAV TLSALIYCLR CLVGRDIPLN
1060 1070 1080 1090 1100
QGCLAPVRVV IPRGSILDPS PEAAVVGGNV LTSQRVVDVI LGAFGACAAS
1110 1120 1130 1140 1150
QGCMNNVTLG NAHMGYYETV AGGAGAGPSW HGRSGVHSHM TNTRITDPEI
1160 1170 1180 1190 1200
LESRYPVILR RFELRRGSGG RGRFRGGDGV TRELLFREEA LLSVLTERRA
1210 1220 1230 1240 1250
FRPYGLHGGE PGARGLNLLI RKNGRTVNLG GKTSVTVYPG DVFCLHTPGG
1260 1270 1280
GGYGDPEDPA PPPGSPPQAL AFPEHGSVYE YRRAQEAV
Computationally mapped potential isoform sequencesi
There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basketA0A087WY99 | A0A087WY99_HUMAN | 5-oxoprolinase | OPLAH | 253 | Annotation score: |
Sequence cautioni
The sequence AAB81519 differs from that shown. Reason: Frameshift.Curated
The sequence CAB46426 differs from that shown. Reason: Frameshift.Curated
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 1084 – 1089 | QRVVDV → NAWWMF in AAB81519 (Ref. 4) Curated | 6 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_050425 | 284 | S → R. Corresponds to variant dbSNP:rs3935209Ensembl. | 1 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AB122018 mRNA Translation: BAD13434.1 BC142672 mRNA Translation: AAI42673.1 AL096750 mRNA Translation: CAB46426.2 Frameshift. AH005594 Genomic DNA Translation: AAB81519.1 Frameshift. |
CCDSi | CCDS75802.1 |
PIRi | T12537 |
RefSeqi | NP_060040.1, NM_017570.4 |
Genome annotation databases
Ensembli | ENST00000618853; ENSP00000480476; ENSG00000178814 |
GeneIDi | 26873 |
KEGGi | hsa:26873 |
MANE-Selecti | ENST00000618853.5; ENSP00000480476.1; NM_017570.5; NP_060040.1 |
UCSCi | uc033cce.2, human |
Similar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AB122018 mRNA Translation: BAD13434.1 BC142672 mRNA Translation: AAI42673.1 AL096750 mRNA Translation: CAB46426.2 Frameshift. AH005594 Genomic DNA Translation: AAB81519.1 Frameshift. |
CCDSi | CCDS75802.1 |
PIRi | T12537 |
RefSeqi | NP_060040.1, NM_017570.4 |
3D structure databases
SMRi | O14841 |
ModBasei | Search... |
Protein-protein interaction databases
BioGRIDi | 117922, 10 interactors |
IntActi | O14841, 5 interactors |
MINTi | O14841 |
STRINGi | 9606.ENSP00000480476 |
Chemistry databases
DrugBanki | DB00142, Glutamic acid |
PTM databases
GlyGeni | O14841, 1 site, 1 O-linked glycan (1 site) |
iPTMneti | O14841 |
MetOSitei | O14841 |
PhosphoSitePlusi | O14841 |
Genetic variation databases
BioMutai | OPLAH |
Proteomic databases
EPDi | O14841 |
jPOSTi | O14841 |
MassIVEi | O14841 |
MaxQBi | O14841 |
PeptideAtlasi | O14841 |
PRIDEi | O14841 |
ProteomicsDBi | 48272 |
Protocols and materials databases
Antibodypediai | 7618, 71 antibodies from 21 providers |
DNASUi | 26873 |
Genome annotation databases
Ensembli | ENST00000618853; ENSP00000480476; ENSG00000178814 |
GeneIDi | 26873 |
KEGGi | hsa:26873 |
MANE-Selecti | ENST00000618853.5; ENSP00000480476.1; NM_017570.5; NP_060040.1 |
UCSCi | uc033cce.2, human |
Organism-specific databases
CTDi | 26873 |
DisGeNETi | 26873 |
GeneCardsi | OPLAH |
HGNCi | HGNC:8149, OPLAH |
HPAi | ENSG00000178814, Tissue enhanced (testis) |
MalaCardsi | OPLAH |
MIMi | 260005, phenotype 614243, gene |
neXtProti | NX_O14841 |
OpenTargetsi | ENSG00000178814 |
Orphaneti | 33572, 5-oxoprolinase deficiency |
PharmGKBi | PA31935 |
VEuPathDBi | HostDB:ENSG00000178814 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG1939, Eukaryota |
GeneTreei | ENSGT00390000013463 |
HOGENOMi | CLU_002157_0_0_1 |
InParanoidi | O14841 |
OMAi | MVVTKGF |
OrthoDBi | 62698at2759 |
PhylomeDBi | O14841 |
Enzyme and pathway databases
BRENDAi | 3.5.2.9, 2681 |
PathwayCommonsi | O14841 |
Reactomei | R-HSA-174403, Glutathione synthesis and recycling R-HSA-5578998, Defective OPLAH causes OPLAHD |
SignaLinki | O14841 |
Miscellaneous databases
BioGRID-ORCSi | 26873, 6 hits in 209 CRISPR screens |
GenomeRNAii | 26873 |
Pharosi | O14841, Tbio |
PROi | PR:O14841 |
RNActi | O14841, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000178814, Expressed in heart left ventricle and 174 other tissues |
ExpressionAtlasi | O14841, baseline and differential |
Genevisiblei | O14841, HS |
Family and domain databases
InterProi | View protein in InterPro IPR008040, Hydant_A_N IPR002821, Hydantoinase_A IPR003692, Hydantoinase_B IPR045079, Oxoprolinase_fam |
PANTHERi | PTHR11365, PTHR11365, 1 hit |
Pfami | View protein in Pfam PF05378, Hydant_A_N, 1 hit PF01968, Hydantoinase_A, 1 hit PF02538, Hydantoinase_B, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | OPLA_HUMAN | |
Accessioni | O14841Primary (citable) accession number: O14841 Secondary accession number(s): A5PKY8, Q75W65, Q9Y4Q0 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | December 15, 1998 |
Last sequence update: | February 15, 2005 | |
Last modified: | February 23, 2022 | |
This is version 161 of the entry and version 3 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any 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. |
Miscellaneousi
Keywords - Technical termi
Reference proteomeDocuments
- Human chromosome 8
Human chromosome 8: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - SIMILARITY comments
Index of protein domains and families