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Serine
hydroxymethyltransferase,
cytosolic
Methylenetetrahydrofolate
reductase
Methionine
synthase
Cystathionine
gamma-lyase
Cystathionine
beta-synthase
Cystathionine
beta-synthase
Betaine--
homocysteine S-
methyltransferase
1
S-
adenosylmethionine
decarboxylase
proenzyme
Spermidine
synthase
S-methyl-5'-
thioadenosine
phosphorylase
Adenosylhomocysteinase
Betaine--
homocysteine S-
methyltransferase
1
L-amino-acid
oxidase
Methionine--
tRNA ligase,
cytoplasmic
Methionyl-tRNA
formyltransferase,
mitochondrial
S-
adenosylmethionine
synthase
Choline
dehydrogenase,
mitochondrial
Methionine-R-
sulfoxide
reductase B3
Methionine-R-
sulfoxide
reductase B2,
mitochondrial
Glycine N-
methyltransferase
L-Homoserine
L-Cysteine
Homocysteine
Tetrahydrofolic acid
L-Serine
5,10-Methylene-THF
Glycine
5-Methyltetrahydrofolic acid
L-Methionine
L-Cystathionine
H
2
O
2-Ketobutyric acid
L-Serine
H
2
O
L-Serine
H
2
O
S-Adenosylmethionine
S-Adenosylmethioninamine
CO
2
Putrescine
5'-Methylthioadenosine
Spermidine
P
i
5-Methylthioribose 1-phosphate
S-Adenosylhomocysteine
Adenosine
βine
Dimethylglycine
H
2
O
O
2
2-Oxo-4-methylthiobutanoic
acid
NH
3
H
2
O
2
ATP
AMP
PP
i
10-Formyltetrahydrofolate
Tetrahydrofolic acid
ATP
P
i
PP
i
Choline
Methionine sulfoxide
Sarcosine
Glycine
Pyridoxal
5'-phosphate
Zinc (II) ion
Pyridoxal
5'-phosphate
Pyridoxal
5'-phosphate
Zinc (II) ion
FAD
L-Methionine
Acceptor
Reduced
acceptor
oxidized
thioredoxin
reduced
thioredoxin
tRNA(Met)
L-
Methionyl-
tRNA(Met)
N-
formylmethionyl-
tRNA(fMet)
Glycine and
Serine
Metabolism
Cysteine
Metabolism
Sulfate/Sulfite
Metabolism
FAD
Pyridoxal
5'-phosphate
Zinc (II) ion
NAD
Magnesium
Potassium
Zinc
Zinc
FAD
Intracellular space
Nuclear envelope
Mitochondria
Mitochondria
Symptoms include
megaloblastic anemia,
developmental delays,
seisures and kidney failure.
Brain
Red Blood Cell
Kidney