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Methionine Metabolism
smpdb.ca/view/SMP0000033Metabolicin a methylated product plus S-adenosylhomocysteine, and the conversion of S-adenosylhomocysteine to produce the compounds homocysteine and adenosine. … These pathways have three common reactions with both pathways including the transformation of methionine to S-adenosylmethionine (SAM), the use of SAM in many different transmethylation reactions resulting
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: S-adenosylmethionine decarboxylase proenzyme, S-methyl-5'-thioadenosine phosphorylaseMethylenetetrahydrofolate Reductase Deficiency (MTHFRD)
smpdb.ca/view/SMP0000340DiseaseMethylenetetrahydrofolate reductase deficiency (MTHFRD; Homocystinuria due to defect of n(5,10)-methylene THF deficiency) is caused by a defect in the MTHFR gene which codes for methylenetetrahydrofolate reductase. Methylenetetrahydrofol...
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: S-adenosylmethionine decarboxylase proenzyme, S-methyl-5'-thioadenosine phosphorylaseHomocystinuria-Megaloblastic Anemia Due to Defect in Cobalamin Metabolism, cblG Complementation Type
smpdb.ca/view/SMP0125684DiseaseHomocystinuria-megaloblastic anemia due to defect in cobalamin metabolism, cblG complementation type, also known as methionine synthase deficiency or methylcobalamin deficiency, cblG type, is a rare inborn error of metabolism (IEM) and a...
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: S-adenosylmethionine decarboxylase proenzyme, S-methyl-5'-thioadenosine phosphorylaseMigalastat
go.drugbank.com/drugs/DB05018ApprovedInvestigational[L47036,L47057] As of August 2018, migalastat under Amicus Therapeutics' brand name Galafold is currently approved in Australia, Canada, European Union, Israel, Japan, South Korea, Switzerland, and … Fabry disease is a rare, progressive genetic disorder characterized by a defective GLA gene that causes a deficiency in the enzyme alpha-Galactosidase A (alpha-Gal A).
Synonyms: (2R,3S,4R,5S)-2-(Hydroxymethyl)piperidine-3,4,5-triol, 1-DeoxygalactostatinCategories: Alpha-Galactosidase A (alpha-Gal A) Pharmacological Chaperones, Heterocyclic Compounds, 1-RingGlycine N-Methyltransferase Deficiency
smpdb.ca/view/SMP0000222DiseaseGNMT catalyzes the conversion of glycine into N-methylglycine (sarcosine) using S-adenosylmethionine (SAM or AdoMet).
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: S-adenosylmethionine decarboxylase proenzyme, S-methyl-5'-thioadenosine phosphorylaseHomocystinuria-Megaloblastic Anemia Due to Defect in Cobalamin Metabolism, cblG Complementation Type
smpdb.ca/view/SMP0000570DiseaseHomocystinuria-megaloblastic anemia due to defect in cobalamin metabolism, cblG complementation type, also known as methionine synthase deficiency or methylcobalamin deficiency, cblG type, is a rare inborn error of metabolism (IEM) and a...
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: S-adenosylmethionine decarboxylase proenzyme, S-methyl-5'-thioadenosine phosphorylaseNitric oxide synthase
go.drugbank.com/bio_entities/BE0010197TargetEnzymeProbably has nitrosylase activity and mediates cysteine S-nitrosylation of cytoplasmic target proteins such SRR … Also has nitrosylase activity and mediates cysteine S-nitrosylation of cytoplasmic target proteins such PTGS2/COX2 (By similarity).
Synonyms: Peptidyl-cysteine S-nitrosylase NOS1, Peptidyl-cysteine S-nitrosylase NOS2Betaine Metabolism
smpdb.ca/view/SMP0000123MetabolicIn the liver, betaine functions as a methyl donor similar to choline, folic acid, S-adenosyl methionine and vitamin B12.
Enzymes: S-adenosylmethionine synthase isoform type-2, Betaine--homocysteine S-methyltransferase 1Methionine 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 phosphorylase