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S-Adenosylhomocysteine (SAH) Hydrolase Deficiency
smpdb.ca/view/SMP0125680DiseaseAHCY codes for the enzyme S-adenosylhomocysteine hydrolase (AdoHcyase) which efficiently eliminates S-adenosylhomocysteine (SAH) by catalyzing its hydrolysis into adenosine and homocysteine. … SAH is both a byproduct of S-adenosylmethionine-dependent methyltransferases and a powerful methyltransferase inhibitor.
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: S-adenosylmethionine decarboxylase proenzyme, S-methyl-5'-thioadenosine phosphorylaseS-Adenosylhomocysteine (SAH) Hydrolase Deficiency
smpdb.ca/view/SMP0000214DiseaseAHCY codes for the enzyme S-adenosylhomocysteine hydrolase (AdoHcyase) which efficiently eliminates S-adenosylhomocysteine (SAH) by catalyzing its hydrolysis into adenosine and homocysteine. … SAH is both a byproduct of S-adenosylmethionine-dependent methyltransferases and a powerful methyltransferase inhibitor.
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: S-adenosylmethionine decarboxylase proenzyme, S-methyl-5'-thioadenosine phosphorylaseMetabolism and Physiological Effects of S-Adenosylhomocysteine
smpdb.ca/view/SMP0127690MetabolicS-Adenosyl-L-homocysteine (SAH) is formed by the demethylation of S-adenosyl-L-methionine. … S-Adenosylhomocysteine forms when there are elevated levels of homocysteine and adenosine. S-Adenosyl-L-homocysteine is a potent inhibitor of S-adenosyl-L-methionine-dependent methylation reactions.
Enzymes: S-methylmethionine--homocysteine S-methyltransferase BHMT2, S-adenosylmethionine synthase isoform type-2Spermidine and Spermine Biosynthesis
smpdb.ca/view/SMP0000445MetabolicSpermidine is subsequently processed into spermine by spermine synthase in the presence of the aminopropyl donor, S-adenosylmethioninamine. … Spermidine originates from the action of spermidine synthase, which converts the methionine derivative S-adenosylmethionine and the ornithine derivative putrescine into spermidine 5'-methylthioadenosine
Enzymes: S-adenosylmethionine decarboxylase proenzyme, S-adenosylmethionine synthase isoform type-2Methionine 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 phosphorylaseHypermethioninemia
smpdb.ca/view/SMP0000341DiseaseThis gene is responsible for Adenosylhomocysteinase, an enzyme which takes S-adenosyl homocysteine as input, and produces homocysteine as its output.
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: S-adenosylmethionine decarboxylase proenzyme, S-methyl-5'-thioadenosine phosphorylaseGlycine 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 phosphorylaseGlycine N-Methyltransferase Deficiency
smpdb.ca/view/SMP0125681DiseaseGNMT 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 phosphorylaseBetaine 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 1Hypermethioninemia
smpdb.ca/view/SMP0125679DiseaseThis gene is responsible for Adenosylhomocysteinase, an enzyme which takes S-adenosyl homocysteine as input, and produces homocysteine as its output.
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 moreEnzymes: S-adenosylmethionine decarboxylase proenzyme, S-methyl-5'-thioadenosine phosphorylase