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Metabolism and Physiological Effects of N-Formyl-L-Methionine
smpdb.ca/view/SMP0126765MetabolicThe conversion of methionine to N-N-Formyl-L-Methionine (L-methionyl-tRNAMet) is completed in the mitochondria by Methionine--tRNA ligase (MARS2). … N-Formyl-L-methionine belongs to the class of organic compounds known as methionine and derivatives.
Enzymes: Methionine--tRNA ligase, mitochondrialLevomefolic acid
go.drugbank.com/drugs/DB11256Approvedincluding the blood-brain barrier into various tissues where it plays an essential role in the DNA synthesis, cysteine cycle and regulation of homocysteine, where it methylates homocysteine and forms methionine
Betaine
go.drugbank.com/drugs/DB06756ApprovedInvestigationalNutraceuticalBetaine is a methyl group donor that functions in the normal metabolic cycle of methionine.
Folic acid
go.drugbank.com/drugs/DB00158ApprovedInvestigationalNutraceuticalVet approvedMore specifically, folic acid is required by the body for the synthesis of purines, pyrimidines, and methionine before incorporation into DNA or protein.
Ademetionine
go.drugbank.com/drugs/DB00118ApprovedInvestigationalNutraceuticalPhysiologic methyl radical donor involved in enzymatic transmethylation reactions and present in all living organisms. It possesses anti-inflammatory activity and has been used in treatment of chronic liver disease. (From Merck, 11th ed)
Cystathionine beta-Synthase Deficiency
smpdb.ca/view/SMP0000177DiseaseTreatment includes folic acid, vitamin B6, vitamin B12, and a methionine-restricted diet. … A deficiency in this enzyme results in accumulation of L-cystathionine, homocysteine, and L-homocystine in plasma and urine; and L-methionine and ornithine in plasma.
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: Methionine synthase, Methionine--tRNA ligase, cytoplasmicHypermethioninemia
smpdb.ca/view/SMP0000341DiseaseThis outputted compound through the its respective pathway may be turned back into cysteine methionine.
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: Methionine synthase, Methionine--tRNA ligase, cytoplasmicS-Adenosylhomocysteine (SAH) Hydrolase Deficiency
smpdb.ca/view/SMP0125680DiseaseAdoHcyase deficiency causes a buildup of homocysteine which may be then converted into methionine or cysteine. … The accumulation of methionine as a result of AHCY deficiency may lead to signs and symptoms associated with hypermethioninemia, including mental and motor retardation, dysmorphism (unusual facial features
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: Methionine synthase, Methionine--tRNA ligase, cytoplasmicMethylenetetrahydrofolate Reductase Deficiency (MTHFRD)
smpdb.ca/view/SMP0000340DiseaseA defect in this enzyme results in accumulation of homocysteine and methionine in both plasma and urine. … Methylenetetrahydrofolate reductase catalyzes the conversion of 5,10-methylenetetrahydrofolate to 5-methyltetrahydrofolate, a co-substrate for homocysteine remethylation to methionine.
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: Methionine synthase, Methionine--tRNA ligase, cytoplasmicHomocystinuria-Megaloblastic Anemia Due to Defect in Cobalamin Metabolism, cblG Complementation Type
smpdb.ca/view/SMP0125684DiseaseMethionine synthase deficiency is characterized by an increase in homocysteine levels in the body and excreted in the urine, as well as decreased levels of methionine in the blood. … It is caused by a mutation in the MTR gene which encodes the enzyme methionine synthase.
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: Methionine synthase, Methionine--tRNA ligase, cytoplasmic