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Sodium-coupled neutral amino acid transporter 4 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 L-Methionine L-Methionine Tetrahydrofolic acid L-Serine 5,10-Methylene-THF Glycine 5-Methyltetrahydrofolic acid L-Cystathionine H2O 2-Ketobutyric acid L-Serine H2O L-Serine H2O S-Adenosylmethionine S-Adenosylmethioninamine CO2 Putrescine 5'-Methylthioadenosine Spermidine Pi 5-Methylthioribose 1-phosphate L-Methionine S-Adenosylhomocysteine Adenosine βine Dimethylglycine H2O O2 2-Oxo-4-methylthiobutanoic acid NH3 H2O2 ATP AMP PPi 10-Formyltetrahydrofolate Tetrahydrofolic acid ATP Pi PPi Choline Methionine sulfoxide Sarcosine Glycine Pyridoxal 5'-phosphate Zinc (II) ion Pyridoxal 5'-phosphate Pyridoxal 5'-phosphate Zinc (II) ion FAD 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 dystonia, mental retardation and unusual odor.
Mitochondria SLC38A4 SHMT1 MTHFR MTR CTH CBS CBS BHMT AMD1 SRM MTAP AHCY BHMT IL4I1 MARS MTFMT MAT2A MAT2B CHDH MSRB3 MSRB2 GNMT L-Homoserine L-Cysteine Homocysteine L-Methionine L-Methionine Tetrahydrofolic acid L-Serine 5,10- Methylene-THF Glycine 5- Methyltetrahydrofolic acid L-Cystathionine Water 2-Ketobutyric acid L-Serine Water L-Serine Water S- Adenosylmethionine S- Adenosylmethioninamine Carbon dioxide Putrescine 5'- Methylthioadenosine Spermidine Phosphate 5- Methylthioribose 1-phosphate L-Methionine S- Adenosylhomocysteine Adenosine βine Dimethylglycine Water Oxygen 2-Oxo-4- methylthiobutanoic acid Ammonia Hydrogen peroxide Adenosine triphosphate Adenosine monophosphate Pyrophosphate 10- Formyltetrahydrofolate Tetrahydrofolic acid Adenosine triphosphate Phosphate Pyrophosphate Choline Methionine sulfoxide Sarcosine Glycine 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
SLC38A4 SHMT1 MTHFR MTR CTH CBS CBS BHMT AMD1 SRM MTAP AHCY BHMT IL4I1 MARS MTFMT MAT2A MAT2B CHDH MSRB3 MSRB2 GNMT Homoser Cys Hcys Met Met THFA Ser 5XM-THF Gly 5-MTHFa L-Cystt H2O 2KBA Ser H2O Ser H2O SAMe Dadomet CO2 Putrsce MTA Spermd Pi 5MTR1P Met SAH Adenosi βine DMglyc H2O O2 KMTB NH3 H2O2 ATP AMP PPi 10-FTHF THFA ATP Pi PPi Choline Met-Sof Sar Gly Pyr-5'P Zinc Pyr-5'P Pyr-5'P Zinc FAD Accepto RA OT RT TRNAMET LMTM NFTF PW000157 PW000018 PW000040 FAD Pyr-5'P Zinc NAD Mg2+ K+ Zinc Zinc FAD Intracellular space Nuclear envelope Mitochondria Mitochondria Symptoms include dystonia, mental retardation and unusual odor.
Mitochondria SLC38A4 SHMT1 MTHFR MTR CTH CBS CBS BHMT AMD1 SRM MTAP AHCY BHMT IL4I1 MARS MTFMT MAT2A MAT2B CHDH MSRB3 MSRB2 GNMT Homoser Cys Hcys Met Met THFA Ser 5XM-THF Gly 5-MTHFa L-Cystt H2O 2KBA Ser H2O Ser H2O SAMe Dadomet CO2 Putrsce MTA Spermd Pi 5MTR1P Met SAH Adenosi βine DMglyc H2O O2 KMTB NH3 H2O2 ATP AMP Ppi 10-FTHF THFA ATP Pi Ppi Choline Met-Sof Sar Gly Accepto RA OT RT TRNAMET LMTM NFTF PW000157 PW000018 PW000040