Details for: OPLAH

Gene ID: 26873

Symbol: OPLAH

Ensembl ID: ENSG00000178814

Description: 5-oxoprolinase, ATP-hydrolysing

Associated with

Cells (max top 100)

(Cell Significance Index and respective Thresholds are uniquely calculated using our advanced thresholding algorithms to reveal cell-specific gene markers)

  • Cell Name: polychromatophilic erythroblast (CL0000550)
    Fold Change: 43.7172
    Cell Significance Index: -6.8000
  • Cell Name: hematopoietic oligopotent progenitor cell (CL0002032)
    Fold Change: 27.0064
    Cell Significance Index: -6.8500
  • Cell Name: mucosal type mast cell (CL0000485)
    Fold Change: 14.8674
    Cell Significance Index: -6.0400
  • Cell Name: ciliated cell of the bronchus (CL0002332)
    Fold Change: 6.3158
    Cell Significance Index: -6.0300
  • Cell Name: enteroendocrine cell of colon (CL0009042)
    Fold Change: 2.4099
    Cell Significance Index: 458.6100
  • Cell Name: stromal cell of bone marrow (CL0010001)
    Fold Change: 1.7334
    Cell Significance Index: -6.8400
  • Cell Name: enterocyte of epithelium of large intestine (CL0002071)
    Fold Change: 1.4865
    Cell Significance Index: 67.3800
  • Cell Name: paneth cell of epithelium of small intestine (CL1000343)
    Fold Change: 1.1258
    Cell Significance Index: 24.3900
  • Cell Name: tuft cell of colon (CL0009041)
    Fold Change: 0.9797
    Cell Significance Index: 884.6400
  • Cell Name: enterocyte of epithelium of small intestine (CL1000334)
    Fold Change: 0.8474
    Cell Significance Index: 24.4200
  • Cell Name: gut absorptive cell (CL0000677)
    Fold Change: 0.7492
    Cell Significance Index: 44.9800
  • Cell Name: intestinal crypt stem cell of colon (CL0009043)
    Fold Change: 0.7347
    Cell Significance Index: 79.9200
  • Cell Name: epithelial cell of small intestine (CL0002254)
    Fold Change: 0.6458
    Cell Significance Index: 105.0400
  • Cell Name: sebaceous gland cell (CL2000021)
    Fold Change: 0.5488
    Cell Significance Index: 0.7100
  • Cell Name: epithelial cell of sweat gland (CL1000448)
    Fold Change: 0.5177
    Cell Significance Index: 0.3700
  • Cell Name: cardiac muscle myoblast (CL0000513)
    Fold Change: 0.4580
    Cell Significance Index: 35.1500
  • Cell Name: microfold cell of epithelium of small intestine (CL1000353)
    Fold Change: 0.3945
    Cell Significance Index: 27.2800
  • Cell Name: basal epithelial cell of tracheobronchial tree (CL0002329)
    Fold Change: 0.3500
    Cell Significance Index: 9.7800
  • Cell Name: periportal region hepatocyte (CL0019026)
    Fold Change: 0.2923
    Cell Significance Index: 4.3200
  • Cell Name: colon goblet cell (CL0009039)
    Fold Change: 0.2346
    Cell Significance Index: 23.2100
  • Cell Name: epithelial cell of stomach (CL0002178)
    Fold Change: 0.2258
    Cell Significance Index: 26.3100
  • Cell Name: decidual cell (CL2000002)
    Fold Change: 0.1973
    Cell Significance Index: 3.1700
  • Cell Name: transit amplifying cell of small intestine (CL0009012)
    Fold Change: 0.1731
    Cell Significance Index: 3.5900
  • Cell Name: type I muscle cell (CL0002211)
    Fold Change: 0.1402
    Cell Significance Index: 3.4200
  • Cell Name: intestinal crypt stem cell of small intestine (CL0009017)
    Fold Change: 0.1334
    Cell Significance Index: 2.8400
  • Cell Name: regular atrial cardiac myocyte (CL0002129)
    Fold Change: 0.1298
    Cell Significance Index: 1.7500
  • Cell Name: epithelial cell of esophagus (CL0002252)
    Fold Change: 0.1265
    Cell Significance Index: 0.8400
  • Cell Name: foveolar cell of stomach (CL0002179)
    Fold Change: 0.1235
    Cell Significance Index: 0.8100
  • Cell Name: basal cell of prostate epithelium (CL0002341)
    Fold Change: 0.1227
    Cell Significance Index: 3.3400
  • Cell Name: bladder urothelial cell (CL1001428)
    Fold Change: 0.1101
    Cell Significance Index: 5.7200
  • Cell Name: neoplastic cell (CL0001063)
    Fold Change: 0.1071
    Cell Significance Index: 21.2600
  • Cell Name: small intestine goblet cell (CL1000495)
    Fold Change: 0.1036
    Cell Significance Index: 3.6400
  • Cell Name: acinar cell of salivary gland (CL0002623)
    Fold Change: 0.0935
    Cell Significance Index: 4.3600
  • Cell Name: sebum secreting cell (CL0000317)
    Fold Change: 0.0895
    Cell Significance Index: 6.3300
  • Cell Name: GABAergic interneuron (CL0011005)
    Fold Change: 0.0864
    Cell Significance Index: 59.7400
  • Cell Name: cardiac muscle cell (CL0000746)
    Fold Change: 0.0711
    Cell Significance Index: 1.0500
  • Cell Name: intermediate cell of urothelium (CL4030055)
    Fold Change: 0.0598
    Cell Significance Index: 10.7800
  • Cell Name: basal cell of urothelium (CL1000486)
    Fold Change: 0.0516
    Cell Significance Index: 6.3400
  • Cell Name: enteroendocrine cell of small intestine (CL0009006)
    Fold Change: 0.0448
    Cell Significance Index: 1.1200
  • Cell Name: hair follicular keratinocyte (CL2000092)
    Fold Change: 0.0445
    Cell Significance Index: 19.6700
  • Cell Name: paneth cell of colon (CL0009009)
    Fold Change: 0.0394
    Cell Significance Index: 0.5900
  • Cell Name: stromal cell of ovary (CL0002132)
    Fold Change: 0.0288
    Cell Significance Index: 3.9500
  • Cell Name: odontoblast (CL0000060)
    Fold Change: 0.0256
    Cell Significance Index: 3.2800
  • Cell Name: cell in vitro (CL0001034)
    Fold Change: 0.0152
    Cell Significance Index: 8.3000
  • Cell Name: lactocyte (CL0002325)
    Fold Change: 0.0148
    Cell Significance Index: 1.9100
  • Cell Name: kidney loop of Henle cortical thick ascending limb epithelial cell (CL1001109)
    Fold Change: 0.0098
    Cell Significance Index: 7.2200
  • Cell Name: pigmented epithelial cell (CL0000529)
    Fold Change: 0.0035
    Cell Significance Index: 6.6600
  • Cell Name: progenitor cell of mammary luminal epithelium (CL0009116)
    Fold Change: -0.0019
    Cell Significance Index: -0.1400
  • Cell Name: non-pigmented ciliary epithelial cell (CL0002304)
    Fold Change: -0.0023
    Cell Significance Index: -1.4600
  • Cell Name: epithelial cell of pancreas (CL0000083)
    Fold Change: -0.0027
    Cell Significance Index: -0.0400
  • Cell Name: anterior lens cell (CL0002223)
    Fold Change: -0.0027
    Cell Significance Index: -4.9900
  • Cell Name: pulmonary alveolar epithelial cell (CL0000322)
    Fold Change: -0.0040
    Cell Significance Index: -3.0000
  • Cell Name: lens epithelial cell (CL0002224)
    Fold Change: -0.0042
    Cell Significance Index: -6.4300
  • Cell Name: pancreatic acinar cell (CL0002064)
    Fold Change: -0.0044
    Cell Significance Index: -0.7500
  • Cell Name: transit amplifying cell of colon (CL0009011)
    Fold Change: -0.0056
    Cell Significance Index: -0.1800
  • Cell Name: secondary lens fiber (CL0002225)
    Fold Change: -0.0063
    Cell Significance Index: -8.5800
  • Cell Name: pancreatic A cell (CL0000171)
    Fold Change: -0.0101
    Cell Significance Index: -7.5000
  • Cell Name: eye photoreceptor cell (CL0000287)
    Fold Change: -0.0103
    Cell Significance Index: -0.6500
  • Cell Name: pancreatic ductal cell (CL0002079)
    Fold Change: -0.0110
    Cell Significance Index: -1.2600
  • Cell Name: ciliary muscle cell (CL1000443)
    Fold Change: -0.0116
    Cell Significance Index: -5.2900
  • Cell Name: type B pancreatic cell (CL0000169)
    Fold Change: -0.0127
    Cell Significance Index: -7.1400
  • Cell Name: abnormal cell (CL0001061)
    Fold Change: -0.0132
    Cell Significance Index: -1.3500
  • Cell Name: pancreatic PP cell (CL0002275)
    Fold Change: -0.0135
    Cell Significance Index: -8.4600
  • Cell Name: obsolete caudal ganglionic eminence derived GABAergic cortical interneuron (CL4023070)
    Fold Change: -0.0147
    Cell Significance Index: -5.2800
  • Cell Name: luminal adaptive secretory precursor cell of mammary gland (CL4033057)
    Fold Change: -0.0160
    Cell Significance Index: -0.7500
  • Cell Name: pigmented ciliary epithelial cell (CL0002303)
    Fold Change: -0.0206
    Cell Significance Index: -2.9900
  • Cell Name: dopaminergic neuron (CL0000700)
    Fold Change: -0.0248
    Cell Significance Index: -7.1300
  • Cell Name: L2/3-6 intratelencephalic projecting glutamatergic neuron (CL4023040)
    Fold Change: -0.0252
    Cell Significance Index: -5.0600
  • Cell Name: subcutaneous adipocyte (CL0002521)
    Fold Change: -0.0276
    Cell Significance Index: -0.1200
  • Cell Name: pancreatic D cell (CL0000173)
    Fold Change: -0.0373
    Cell Significance Index: -7.8600
  • Cell Name: epidermal cell (CL0000362)
    Fold Change: -0.0399
    Cell Significance Index: -0.0900
  • Cell Name: helper T cell (CL0000912)
    Fold Change: -0.0450
    Cell Significance Index: -0.6400
  • Cell Name: kidney loop of Henle descending limb epithelial cell (CL1001021)
    Fold Change: -0.0484
    Cell Significance Index: -3.8300
  • Cell Name: fibro/adipogenic progenitor cell (CL0009099)
    Fold Change: -0.0510
    Cell Significance Index: -2.5800
  • Cell Name: epithelial cell of uterus (CL0002149)
    Fold Change: -0.0512
    Cell Significance Index: -0.7100
  • Cell Name: tonsil germinal center B cell (CL2000006)
    Fold Change: -0.0587
    Cell Significance Index: -6.9200
  • Cell Name: conjunctival epithelial cell (CL1000432)
    Fold Change: -0.0594
    Cell Significance Index: -0.8100
  • Cell Name: smooth muscle cell of sphincter of pupil (CL0002243)
    Fold Change: -0.0628
    Cell Significance Index: -6.5400
  • Cell Name: intestinal tuft cell (CL0019032)
    Fold Change: -0.0770
    Cell Significance Index: -4.7200
  • Cell Name: lung endothelial cell (CL1001567)
    Fold Change: -0.0833
    Cell Significance Index: -4.3400
  • Cell Name: glycinergic neuron (CL1001509)
    Fold Change: -0.0884
    Cell Significance Index: -4.6400
  • Cell Name: granulosa cell (CL0000501)
    Fold Change: -0.0974
    Cell Significance Index: -2.5600
  • Cell Name: umbrella cell of urothelium (CL4030056)
    Fold Change: -0.0988
    Cell Significance Index: -0.9100
  • Cell Name: early pro-B cell (CL0002046)
    Fold Change: -0.1045
    Cell Significance Index: -6.7400
  • Cell Name: hippocampal granule cell (CL0001033)
    Fold Change: -0.1047
    Cell Significance Index: -7.0400
  • Cell Name: forebrain neuroblast (CL1000042)
    Fold Change: -0.1093
    Cell Significance Index: -6.7200
  • Cell Name: epithelial cell of nephron (CL1000449)
    Fold Change: -0.1106
    Cell Significance Index: -0.9400
  • Cell Name: skeletal muscle fiber (CL0008002)
    Fold Change: -0.1171
    Cell Significance Index: -3.0100
  • Cell Name: centrilobular region hepatocyte (CL0019029)
    Fold Change: -0.1172
    Cell Significance Index: -1.9800
  • Cell Name: neuron associated cell (CL0000095)
    Fold Change: -0.1188
    Cell Significance Index: -4.8700
  • Cell Name: mesonephric nephron tubule epithelial cell (CL1000022)
    Fold Change: -0.1235
    Cell Significance Index: -4.2900
  • Cell Name: retinal progenitor cell (CL0002672)
    Fold Change: -0.1263
    Cell Significance Index: -7.0900
  • Cell Name: leptomeningeal cell (CL0000708)
    Fold Change: -0.1296
    Cell Significance Index: -2.7700
  • Cell Name: seromucus secreting cell (CL0000159)
    Fold Change: -0.1379
    Cell Significance Index: -2.8800
  • Cell Name: neutrophil progenitor cell (CL0000834)
    Fold Change: -0.1507
    Cell Significance Index: -4.0300
  • Cell Name: slow muscle cell (CL0000189)
    Fold Change: -0.1517
    Cell Significance Index: -2.2700
  • Cell Name: keratocyte (CL0002363)
    Fold Change: -0.1576
    Cell Significance Index: -2.5000
  • Cell Name: indirect pathway medium spiny neuron (CL4023029)
    Fold Change: -0.1597
    Cell Significance Index: -7.0700
  • Cell Name: L5 extratelencephalic projecting glutamatergic cortical neuron (CL4023041)
    Fold Change: -0.1603
    Cell Significance Index: -5.6200
  • Cell Name: stratified epithelial cell (CL0000079)
    Fold Change: -0.1613
    Cell Significance Index: -5.9200

Cell ID: Standard Cell Ontology term used for mapping and comparing cells across experiments. Ensures consistency in analyzing cellular functions across tissues.
Fold Change: Represents the ratio of the current Cell Significance Index to the Cell Significance Index Threshold, indicating how much the gene expression has changed compared to a baseline.
Cell Significance Index: Reflects how strongly a gene is expressed in this specific cell.

Cell ID: Standard Cell Ontology term used for mapping and comparing cells across experiments. Ensures consistency in analyzing cellular functions across tissues.
Fold Change: Represents the ratio of the current Cell Significance Index to the Cell Significance Index Threshold, indicating how much the gene expression has changed compared to a baseline.
Cell Significance Index: Reflects how strongly a gene is expressed in this cell type. Calculated using techniques like effect size estimation and bootstrapping for reliability.

Cell ID: Standard Cell Ontology term used for mapping and comparing cells across experiments. Ensures consistency in analyzing cellular functions across tissues.
Fold Change: Represents the ratio of the current Cell Significance Index to the Cell Significance Index Threshold, indicating how much the gene expression has changed compared to a baseline.
Cell Significance Index: Reflects how strongly a gene is expressed in this cell type. Calculated using techniques like effect size estimation and bootstrapping for reliability.

Other Information

**Key Characteristics:** 1. **5-Oxoprolinase Activity:** OPLAH is a unique enzyme that catalyzes the hydrolysis of 5-oxoprolin, a key intermediate in the glutathione synthesis pathway. 2. **ATP-Hydrolyzing Activity:** OPLAH possesses ATP-hydrolyzing activity, which is essential for its enzymatic function. 3. **Expression in Multiple Tissues:** OPLAH is expressed in various tissues, including cardiac muscle cells, granulocyte monocyte progenitor cells, and cerebral cortex GABAergic interneurons, highlighting its widespread importance in human metabolism. 4. **Regulation of Glutathione Metabolism:** OPLAH is integral to the regulation of glutathione synthesis and recycling, which is critical for maintaining redox balance and preventing oxidative stress. **Pathways and Functions:** 1. **Glutathione Synthesis and Recycling:** OPLAH plays a central role in the glutathione synthesis pathway, facilitating the conversion of 5-oxoprolin to glutathione. 2. **Biological Oxidations:** OPLAH is involved in the regulation of biological oxidations, which is essential for maintaining cellular homeostasis and preventing oxidative damage. 3. **Phase II Conjugation:** OPLAH is also implicated in the phase II conjugation of compounds, a process that involves the conjugation of electrophilic compounds with glutathione or other molecules. 4. **Metabolic Disorders:** Defects in OPLAH have been linked to various metabolic disorders, including oplahd (a rare genetic disorder characterized by impaired glutathione metabolism). **Clinical Significance:** 1. **Oplahd Disease:** OPLAH mutations have been identified as the cause of oplahd, a rare genetic disorder characterized by impaired glutathione metabolism. 2. **Metabolic Disorders:** Defects in OPLAH have been linked to various metabolic disorders, including those related to oxidative stress and glutathione metabolism. 3. **Cardiovascular Disease:** OPLAH expression in cardiac muscle cells suggests that this enzyme may play a role in cardiovascular disease, particularly in the context of impaired glutathione metabolism. 4. **Cancer:** The involvement of OPLAH in the regulation of biological oxidations and phase II conjugation suggests that this enzyme may also play a role in cancer development and progression. In conclusion, the study of OPLAH has provided valuable insights into the regulation of glutathione metabolism and the maintenance of cellular homeostasis. Further research is needed to fully elucidate the role of OPLAH in human health and disease, but the emerging evidence suggests that this enzyme is a critical player in the prevention of metabolic disorders and the promotion of overall health.

Genular Protein ID: 2454890418

Symbol: OPLA_HUMAN

Name: 5-oxoprolinase

UniProtKB Accession Codes:

O14841 A5PKY8 Q75W65 Q9Y4Q0

Database IDs:

AGR 1 AlphaFoldDB 1 Antibodypedia 1 BRENDA 1 Bgee 1 BioCyc 1 BioGRID 1 BioGRID-ORCS 1 BioMuta 1 CCDS 1 CTD 1 ChEBI 7 DNASU 1 DisGeNET 1 DrugBank 1 EC 1 EMBL 4 Ensembl 1 ExpressionAtlas 1 GO 5 GeneCards 1 GeneTree 1 GenomeRNAi 1 GlyGen 1 HGNC 1 HOGENOM 1 HPA 1 InParanoid 1 IntAct 1 InterPro 5 KEGG 1 MANE-Select 1 MIM 3 MINT 1 MalaCards 1 MassIVE 1 MetOSite 1 NCBI Taxonomy 1 OMA 1 OpenTargets 1 Orphanet 1 OrthoDB 1 PANTHER 2 PIR 1 PRO 1 PathwayCommons 1 PaxDb 1 PeptideAtlas 1 Pfam 4 PharmGKB 1 Pharos 1 PhosphoSitePlus 1 PhylomeDB 1 Proteomes 1 ProteomicsDB 1 Pumba 1 RNAct 1 Reactome 2 RefSeq 1 Rhea 1 SMR 1 STRING 1 SignaLink 1 UCSC 1 VEuPathDB 1 eggNOG 1 iPTMnet 1 jPOST 1 neXtProt 1

Citations:

PubMed ID: 14993790

Title: Bovine 5-oxo-L-prolinase: simple assay method, purification, cDNA cloning, and detection of mRNA in the coronary artery.

PubMed ID: 14993790

DOI: 10.1248/bpb.27.288

PubMed ID: 15489334

Title: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).

PubMed ID: 15489334

DOI: 10.1101/gr.2596504

PubMed ID: 17974005

Title: The full-ORF clone resource of the German cDNA consortium.

PubMed ID: 17974005

DOI: 10.1186/1471-2164-8-399

PubMed ID: 21269460

Title: Initial characterization of the human central proteome.

PubMed ID: 21269460

DOI: 10.1186/1752-0509-5-17

PubMed ID: 21651516

Title: 5-Oxoprolinase deficiency: report of the first human OPLAH mutation.

PubMed ID: 21651516

DOI: 10.1111/j.1399-0004.2011.01728.x

PubMed ID: 23186163

Title: Toward a comprehensive characterization of a human cancer cell phosphoproteome.

PubMed ID: 23186163

DOI: 10.1021/pr300630k

PubMed ID: 24275569

Title: An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.

PubMed ID: 24275569

DOI: 10.1016/j.jprot.2013.11.014

PubMed ID: 23430506

Title: 5-Oxoprolinuria in Heterozygous Patients for 5-Oxoprolinase (OPLAH) Missense Changes.

PubMed ID: 23430506

DOI: 10.1007/8904_2012_166

Sequence Information:

  • Length: 1288
  • Mass: 137457
  • Checksum: 999AC0FA497750F2
  • Sequence:
    • MGSPEGRFHF AIDRGGTFTD VFAQCPGGHV RVLKLLSEDP ANYADAPTEG IRRILEQEAG 
MLLPRDQPLD SSHIASIRMG TTVATNALLE RKGERVALLV TRGFRDLLHI GTQARGDLFD 
LAVPMPEVLY EEVLEVDERV VLHRGEAGTG TPVKGRTGDL LEVQQPVDLG ALRGKLEGLL 
SRGIRSLAVV LMHSYTWAQH EQQVGVLARE LGFTHVSLSS EAMPMVRIVP RGHTACADAY 
LTPAIQRYVQ GFCRGFQGQL KDVQVLFMRS DGGLAPMDTF SGSSAVLSGP AGGVVGYSAT 
TYQQEGGQPV IGFDMGGTST DVSRYAGEFE HVFEASTAGV TLQAPQLDIN TVAAGGGSRL 
FFRSGLFVVG PESAGAHPGP ACYRKGGPVT VTDANLVLGR LLPASFPCIF GPGENQPLSP 
EASRKALEAV ATEVNSFLTN GPCPASPLSL EEVAMGFVRV ANEAMCRPIR ALTQARGHDP 
SAHVLACFGG AGGQHACAIA RALGMDTVHI HRHSGLLSAL GLALADVVHE AQEPCSLLYA 
PETFVQLDQR LSRLEEQCVD ALQAQGFPRS QISTESFLHL RYQGTDCALM VSAHQHPATA 
RSPRAGDFGA AFVERYMREF GFVIPERPVV VDDVRVRGTG RSGLRLEDAP KAQTGPPRVD 
KMTQCYFEGG YQETPVYLLA ELGYGHKLHG PCLIIDSNST ILVEPGCQAE VTKTGDICIS 
VGAEVPGTVG PQLDPIQLSI FSHRFMSIAE QMGRILQRTA ISTNIKERLD FSCALFGPDG 
GLVSNAPHIP VHLGAMQETV QFQIQHLGAD LHPGDVLLSN HPSAGGSHLP DLTVITPVFW 
PGQTRPVFYV ASRGHHADIG GITPGSMPPH STMLQQEGAV FLSFKLVQGG VFQEEAVTEA 
LRAPGKVPNC SGTRNLHDNL SDLRAQVAAN QKGIQLVGEL IGQYGLDVVQ AYMGHIQANA 
ELAVRDMLRA FGTSRQARGL PLEVSSEDHM DDGSPIRLRV QISLSQGSAV FDFSGTGPEV 
FGNLNAPRAV TLSALIYCLR CLVGRDIPLN QGCLAPVRVV IPRGSILDPS PEAAVVGGNV 
LTSQRVVDVI LGAFGACAAS QGCMNNVTLG NAHMGYYETV AGGAGAGPSW HGRSGVHSHM 
TNTRITDPEI LESRYPVILR RFELRRGSGG RGRFRGGDGV TRELLFREEA LLSVLTERRA 
FRPYGLHGGE PGARGLNLLI RKNGRTVNLG GKTSVTVYPG DVFCLHTPGG GGYGDPEDPA 
PPPGSPPQAL AFPEHGSVYE YRRAQEAV

Database document:

This is a preview of the gene's schema. Only a few entries are kept for 'singleCellExpressions,' 'mRNAExpressions,' and other large data arrays for visualization purposes. You can zoom in with the mouse wheel for a closer view, and the text will adjust automatically if necessary. For the full schema, download it here.