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Betaine Metabolism
smpdb.ca/view/SMP0000123MetabolicThen, betaine aldehyde dehydrogenase oxidizes betaine aldehyde to betaine in the mitochondria or cytoplasm. … Betaine is obtained from diet as betaine or compounds containing choline in foods such as whole grains, beets and spinach. Betaine can also be synthesized from choline in the liver and kidney.
Enzymes: Betaine--homocysteine S-methyltransferase 1Betaine Drug Metabolism
smpdb.ca/view/SMP0124744MetabolicBetaine is obtained from foods such as wheat, shellfish, spinach, and beets. In the intestine, betaine is metabolized to trimethylamine (TMA) by the gut microbiota (Peptoclostridium acidaminophilum). … TMA is created from betaine via the enzyme betaine reductase in the intestinal microbe, then TMA then enters the bloodstream and is transported to the liver where dimethylaniline monooxygenase [N-oxide-forming
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 1Homocystinuria-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 1Methionine Metabolism
smpdb.ca/view/SMP0000033MetabolicMethionine metabolism is a process that is necessary for humans. Methionine metabolism in mammals happens within two pathways, a methionine cycle and a transsulfuration sequence. These pathways have three common reactions with both pathw...
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: Betaine--homocysteine S-methyltransferase 1