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gamma-Glutamyltranspeptidase Deficiency
smpdb.ca/view/SMP0125618DiseaseGamma-glutamyltranspeptidase deficiency, characterized by glutathionemia and glutathionuria, is an autosomal recessive disorder of glutathione metabolism caused by a defective gamma-glutamyl transpeptidase (GGT). GGT transfers glutamyl m...
Enzymes: Glutathione S-transferase omega-2Methionine Adenosyltransferase Deficiency
smpdb.ca/view/SMP0125683DiseaseMethionine adenosyltransferase (MAT; Hypermethioninemia; MAT I/III deficiency) deficiency is caused by mutations in the MAT1A gene which causes isolated hypermethioninemia. MAT catalyzes the formation of adenosylmethionine from methionin...
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: S-adenosylmethionine decarboxylase proenzyme, S-methyl-5'-thioadenosine phosphorylaseIsoniazid Metabolism
smpdb.ca/view/SMP0121102MetabolicIt can alternatively be processed by cytochrome P450 2E1 into hepatotoxins, which are then joined to glutatione by glutatione S-transferase omega-2 to form R-S-glutatione, which is then excreted.
Enzymes: Glutathione S-transferase omega-2Glutathione Metabolism
smpdb.ca/view/SMP0000015MetabolicGlutathione (GSH) is an low-molecular-weight thiol and antioxidant in various species such as plants, mammals and microbes. Glutathione plays important roles in nutrient metabolism, gene expression, etc. and sufficient protein nutrition ...
Enzymes: Glutathione S-transferase omega-25-Oxoprolinase Deficiency
smpdb.ca/view/SMP0000500Disease5-Oxoprolinase deficiency, also called OPLAHD, is a rare inborn error of metabolism (IEM) and autosomal recessive disorder of glutathione metabolism caused by a defective 5-oxoprolinase. 5-Oxoprolinase catalyzes the conversion of 5-oxopr...
Enzymes: Glutathione S-transferase omega-2gamma-Glutamyltranspeptidase Deficiency
smpdb.ca/view/SMP0000501DiseaseGamma-glutamyltranspeptidase deficiency, characterized by glutathionemia and glutathionuria, is an autosomal recessive disorder of glutathione metabolism caused by a defective gamma-glutamyl transpeptidase (GGT). GGT transfers glutamyl m...
Enzymes: Glutathione S-transferase omega-2Glutathione Synthetase Deficiency
smpdb.ca/view/SMP0125614DiseaseGlutathione Synthetase Deficiency (5-Oxoprolinuria; Pyroglutamic Aciduria; GSD) is a rare inborn error of metabolism (IEM) which arises from a disfunctional gene called GSS. This gene is responsible for glutathione synthetase. Glutathion...
Enzymes: Glutathione S-transferase omega-2gamma-Glutamyltransferase Deficiency
smpdb.ca/view/SMP0125616DiseaseGamma-Glutamyltransferase Deficiency is an autosomal recessive disorder caused by a mutation in the GGT1 gene which codes for gamma-glutamyltranspeptidase 1. A deficiency in this enzyme results in accumulation of L-cysteine, gamma-glutam...
Enzymes: Glutathione S-transferase omega-25-Oxoprolinase Deficiency
smpdb.ca/view/SMP0125617Disease5-Oxoprolinase deficiency, also called OPLAHD, is a rare inborn error of metabolism (IEM) and autosomal recessive disorder of glutathione metabolism caused by a defective 5-oxoprolinase. 5-Oxoprolinase catalyzes the conversion of 5-oxopr...
Enzymes: Glutathione S-transferase omega-2Intracellular Signalling Through PGD2 receptor and Prostaglandin D2
smpdb.ca/view/SMP0000343SignalingThe PGD receptor activates G(s) proteins which lead to the activation of adenylyl cyclase which produces the secondary messenger cAMP. cAMP activates PKA (protein kinase A) which phosphorylates downstream
Drugs: ATP · Magnesium cation · Prostaglandin D2 · Cyclic adenosine monophosphate · Pyrophosphoric acidEnzymes: Guanine nucleotide-binding protein G(s) subunit alpha isoforms short, Guanine nucleotide-binding protein G(I)/G(S)/G(O) subunit gamma-12