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Methionine 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. … When there is a lack of methionine, there is a decrease in the production of SAM, which limits cystathionine synthase activity.
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: Methionine synthase, Methionine--tRNA ligase, cytoplasmicMethionine Adenosyltransferase Deficiency
smpdb.ca/view/SMP0000221DiseaseMAT dificiency is characterized by increased homocysteine and methionine levels in plasma; and accumulation of methionine in urine. Symptoms include dystonia, mental retardation and unusual odor. … MAT catalyzes the formation of adenosylmethionine from methionine and ATP. Adenosylmethionine is an important methyl donor in most transmethylation reactions.
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: Methionine synthase, Methionine--tRNA ligase, cytoplasmicMethionine Adenosyltransferase Deficiency
smpdb.ca/view/SMP0125683DiseaseMAT dificiency is characterized by increased homocysteine and methionine levels in plasma; and accumulation of methionine in urine. Symptoms include dystonia, mental retardation and unusual odor. … MAT catalyzes the formation of adenosylmethionine from methionine and ATP. Adenosylmethionine is an important methyl donor in most transmethylation reactions.
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: Methionine synthase, Methionine--tRNA ligase, cytoplasmicMetabolism 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, mitochondrialCystathionine 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, cytoplasmicS-Adenosylhomocysteine (SAH) Hydrolase Deficiency
smpdb.ca/view/SMP0000214DiseaseAdoHcyase 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, cytoplasmic