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Choline
go.drugbank.com/drugs/DB00122ApprovedInvestigationalNutraceuticalA basic constituent of lecithin that is found in many plants and animal organs. It is important as a precursor of acetylcholine, as a methyl donor in various metabolic processes, and in lipid metabolism.
Metabolism and Physiological Effects of Dimethylglycine
smpdb.ca/view/SMP0124873MetabolicBetaine aldehyde is the converted to betaine through alpha-aminoadipic semialdehyde dehydrogenase. Finally, betaine--homocysteine S-methyltransferase 1 produces dimethylglycine from betaine. … Choline is metabolized in the liver to betaine aldehyde in the mitochondria via the enzyme choline dehydrogenase.
Drugs: Choline · Methionine · NADH · Zinc · N,N-dimethylglycine · Flavin adenine dinucleotide +1 moreSarcosine Oncometabolite Pathway
smpdb.ca/view/SMP0002313DiseaseBetaine aldehyde is then converted to betaine by the addition of a water molecule by alpha-aminoadipic semialdehyde dehydrogenase. … Following this, betaine is transported out of the mitochondria by an unknown transporter, where it then reacts with homocysteine to form dimethylglycine and L-methionine in a reaction catalyzed by betaine-homocysteine
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Serine · Methionine +10 moreEnzymes: Betaine--homocysteine S-methyltransferase 1N,N,N-trimethylglycinium
go.drugbank.com/drugs/DB04455InvestigationalAs a drug, betaine hydrochloride has been used as a source of hydrochloric acid in the treatment of hypochlorhydria. … Betaine has also been used in the treatment of liver disorders, for hyperkalemia, for homocystinuria, and for gastrointestinal disturbances. (From Martindale, The Extra Pharmacopoeia, 30th ed, p1341)
Homocystinuria-Megaloblastic Anemia Due to Defect in Cobalamin Metabolism, cblG Complementation Type
smpdb.ca/view/SMP0125684DiseaseTreatment with vitamin B12, as well as folates and betaine is effective. It is estimated that methionine synthase deficiency affects less than 1 in 1,000,000 individuals.
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: Betaine--homocysteine S-methyltransferase 1Homocystinuria-Megaloblastic Anemia Due to Defect in Cobalamin Metabolism, cblG Complementation Type
smpdb.ca/view/SMP0000570DiseaseTreatment with vitamin B12, as well as folates and betaine is effective. It is estimated that methionine synthase deficiency affects less than 1 in 1,000,000 individuals.
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: Betaine--homocysteine S-methyltransferase 1Cystathionine beta-Synthase Deficiency
smpdb.ca/view/SMP0000177DiseaseCystathionine Beta-Synthase Deficiency (CBS Deficiency; Homocystinuria) is an autosomal recessive disease caused by a mutation in the CBS gene which codes for cystathionine beta-synthase. A deficiency in this enzyme results in accumulati...
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: Betaine--homocysteine S-methyltransferase 1Methionine Adenosyltransferase Deficiency
smpdb.ca/view/SMP0000221DiseaseMethionine 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: Betaine--homocysteine S-methyltransferase 1Hypermethioninemia
smpdb.ca/view/SMP0000341DiseaseHypermethioninemia is a rare error of metabolism (IEM) which arises when there is a disfunction in the gene called AHCY. This gene is responsible for Adenosylhomocysteinase, an enzyme which takes S-adenosyl homocysteine as input, and pro...
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: Betaine--homocysteine S-methyltransferase 1