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OPLAH
Gene Name
5-oxoprolinase (ATP-hydrolysing)
Image
No pdb structure
Gene Ontology Annotations
Cellular Component
Cytosol
Molecular Function
ATP Binding
5-oxoprolinase (ATP-hydrolyzing) Activity
Biological Process
Glutathione Biosynthetic Process
Xenobiotic Metabolic Process
Small Molecule Metabolic Process
Glutathione Derivative Biosynthetic Process
Pathways
Defective OPLAH causes 5-oxoprolinase deficiency (OPLAHD)
Defective AHCY causes Hypermethioninemia with S-adenosylhomocysteine hydrolase deficiency (HMAHCHD)
Defective MAT1A causes Methionine adenosyltransferase deficiency (MATD)
Defective TPMT causes Thiopurine S-methyltransferase deficiency (TPMT deficiency)
Glutathione synthesis and recycling
Defective UGT1A4 causes hyperbilirubinemia
Metabolic disorders of biological oxidation enzymes
Glutathione conjugation
Phase II conjugation
Defective UGT1A1 causes hyperbilirubinemia
Defective SLC35D1 causes Schneckenbecken dysplasia (SCHBCKD)
Biological oxidations
Defective GCLC causes Hemolytic anemia due to gamma-glutamylcysteine synthetase deficiency (HAGGSD)
Defective GGT1 causes Glutathionuria (GLUTH)
Defective GSS causes Glutathione synthetase deficiency (GSS deficiency)
Drugs
Diseases
GWAS
Metabolic traits (
21886157
)
Protein-protein Interactions
1 interactors:
GK
Entrez ID
26873
HPRD ID
14880
Ensembl ID
ENSG00000178814
Uniprot IDs
O14841
PDB IDs
Enriched GO Terms of Interacting Partners
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Glycerol-3-phosphate Biosynthetic Process
Glycerol Catabolic Process
Glycerol-3-phosphate Metabolic Process
Cellular Carbohydrate Catabolic Process
Glycerol Metabolic Process
Triglyceride Biosynthetic Process
Alcohol Catabolic Process
Triglyceride Metabolic Process
Neutral Lipid Metabolic Process
Carbohydrate Catabolic Process
Glucose Homeostasis
Cellular Carbohydrate Metabolic Process
Alcohol Metabolic Process
Glycerolipid Metabolic Process
Lipid Biosynthetic Process
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