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Amine oxidase [flavin- containing] A Aldehyde dehydrogenase, mitochondrial 2-amino-3- ketobutyrate coenzyme A ligase, mitochondrial Dimethylglycine dehydrogenase, mitochondrial Sarcosine dehydrogenase, mitochondrial Glycine dehydrogenase [decarboxylating], mitochondrial Aminomethyltransferase, mitochondrial Serine hydroxymethyltransferase, mitochondrial Dihydrolipoyl dehydrogenase, mitochondrial Serine-- pyruvate aminotransferase Serine-- pyruvate aminotransferase Glycine amidinotransferase, mitochondrial Guanidinoacetate N- methyltransferase 5- aminolevulinate synthase, nonspecific, mitochondrial Betaine-- homocysteine S- methyltransferase 1 Glycerate kinase D-3- phosphoglycerate dehydrogenase Phosphoserine aminotransferase Phosphoserine phosphatase L-serine dehydratase/L- threonine deaminase Serine hydroxymethyltransferase, cytosolic Serine racemase Cystathionine beta-synthase Serine--tRNA ligase, cytoplasmic Glycine--tRNA ligase Cystathionine gamma-lyase Glycine N- methyltransferase L-Cysteine 3-Phosphoglyceric acid Pyruvic acid Pyruvic acid Pyruvic acid L-Serine Aminoacetone O2 H2O NH3 H2O2 Pyruvaldehyde NAD NADH H2O L-2-Amino-3-oxobutanoic acid CO2 Acetyl-CoA Accumulation Glycine CoA Dimethylglycine H2O Formaldehyde Sarcosine H2O Formaldehyde (R)-lipoic acid 8-[(Aminomethyl)sulfanyl]-6- sulfanyloctanoic acid CO2 Dihydrolipoate NH3 5,10-Methylene-THF Accumulation Glycine H2O Tetrahydrofolic acid L-Serine NAD NADH Hydroxypyruvic acid L-Alanine Glyoxylic acid L-Arginine Guanidoacetic acid Orotidylic acid S-Adenosylhomocysteine S-Adenosylmethionine Creatine Succinyl-CoA 5-Aminolevulinic acid CoA βine Homocysteine L-Methionine Glyceric acid ATP ADP NAD Phosphohydroxypyruvic acid NADH L-Glutamic acid Phosphoserine Oxoglutaric acid H2O Pi NH3 Tetrahydrofolic acid Accumulation Glycine 5,10-Methylene-THF H2O D-Serine Homocysteine L-Cystathionine H2O ATP AMP PPi ATP AMP PPi H2O 2-Ketobutyric acid NH3 S-Adenosylhomocysteine S-Adenosylmethionine FAD Pyridoxal 5'-phosphate FAD FAD Pyridoxal 5'-phosphate Pyridoxal 5'-phosphate FAD Pyridoxal 5'-phosphate Pyridoxal 5'-phosphate Magnesium Pyridoxal 5'-phosphate Pyridoxal 5'-phosphate Pyridoxal 5'-phosphate L-Seryl-tRNA L-Seryl- tRNA(Ser) Glycyl-tRNA Glycyl- tRNA(Gly) Cysteine Metabolism Gluconeogenesis Glycolysis Pyruvate Metabolism Pyruvate Metabolism Pyruvate Metabolism Sphingolipid Metabolism Pyridoxal 5'-phosphate Zinc (II) ion Pyridoxal 5'-phosphate Pyridoxal 5'-phosphate Pyridoxal 5'-phosphate Cytosol Mitochondria Outer mitochondria membrane Inner mitochondria membrane Mitochondria matrix
Mitochondria MAOA ALDH2 GCAT DMGDH SARDH GLDC AMT SHMT2 DLD AGXT AGXT GATM GAMT ALAS1 BHMT GLYCTK PHGDH PSAT1 PSPH SDS SHMT1 SRR CBS SARS GARS CTH GNMT L-Cysteine 3- Phosphoglyceric acid Pyruvic acid Pyruvic acid Pyruvic acid L-Serine Aminoacetone Oxygen Water Ammonia Hydrogen peroxide Pyruvaldehyde NAD NADH Water L-2-Amino-3- oxobutanoic acid Carbon dioxide Acetyl-CoA Accumulation Glycine Coenzyme A Dimethylglycine Water Formaldehyde Sarcosine Water Formaldehyde (R)-lipoic acid 8- [(Aminomethyl)sulfanyl]- 6- sulfanyloctanoic acid Carbon dioxide Dihydrolipoate Ammonia 5,10- Methylene-THF Accumulation Glycine Water Tetrahydrofolic acid L-Serine NAD NADH Hydroxypyruvic acid L-Alanine Glyoxylic acid L-Arginine Guanidoacetic acid Orotidylic acid S- Adenosylhomocysteine S- Adenosylmethionine Creatine Succinyl-CoA 5- Aminolevulinic acid Coenzyme A βine Homocysteine L-Methionine Glyceric acid Adenosine triphosphate Adenosine diphosphate NAD Phosphohydroxypyruvic acid NADH L-Glutamic acid Phosphoserine Oxoglutaric acid Water Phosphate Ammonia Tetrahydrofolic acid Accumulation Glycine 5,10- Methylene-THF Water D-Serine Homocysteine L-Cystathionine Water Adenosine triphosphate Adenosine monophosphate Pyrophosphate Adenosine triphosphate Adenosine monophosphate Pyrophosphate Water 2-Ketobutyric acid Ammonia S- Adenosylhomocysteine S- Adenosylmethionine L-Seryl-tRNA L-Seryl- tRNA(Ser) Glycyl-tRNA Glycyl- tRNA(Gly) Cysteine Metabolism Gluconeogenesis Glycolysis Pyruvate Metabolism Pyruvate Metabolism Pyruvate Metabolism Sphingolipid Metabolism
MAOA ALDH2 GCAT DMGDH SARDH GLDC AMT SHMT2 DLD AGXT AGXT GATM GAMT ALAS1 BHMT GLYCTK PHGDH PSAT1 PSPH SDS SHMT1 SRR CBS SARS GARS CTH GNMT Cys G3P Pyr Pyr Pyr Ser Amnactn O2 H2O NH3 H2O2 Pyruval NAD NADH H2O L2AAA CO2 Ac-CoA Accumulation Gly CoA DMglyc H2O Formol Sar H2O Formol Lipoate 8As6SA CO2 Dhlip NH3 5XM-THF Accumulation Gly H2O THFA Ser NAD NADH OH-Pyr Ala Glyoxal Arg GIAA OMP SAH SAMe Creatin Suc-CoA 5-Amnva CoA βine Hcys Met Glycera ATP ADP NAD 3POHPyr NADH Glu SEP AKG H2O Pi NH3 THFA Accumulation Gly 5XM-THF H2O D-Ser Hcys L-Cystt H2O ATP AMP PPi ATP AMP PPi H2O 2KBA NH3 SAH SAMe FAD Pyr-5'P FAD FAD Pyr-5'P Pyr-5'P FAD Pyr-5'P Pyr-5'P Mg2+ Pyr-5'P Pyr-5'P Pyr-5'P LST LSTS GT GTG PW000018 PW000152 PW000146 PW000054 PW000054 PW000054 PW000148 Pyr-5'P Zinc Pyr-5'P Pyr-5'P Pyr-5'P Cytosol Mitochondria Outer mitochondria membrane Inner mitochondria membrane Mitochondria matrix
Mitochondria MAOA ALDH2 GCAT DMGDH SARDH GLDC AMT SHMT2 DLD AGXT AGXT GATM GAMT ALAS1 BHMT GLYCTK PHGDH PSAT1 PSPH SDS SHMT1 SRR CBS SARS GARS CTH GNMT Cys G3P Pyr Pyr Pyr Ser Amnactn O2 H2O NH3 H2O2 Pyruval NAD NADH H2O L2AAA CO2 Ac-CoA Accumulation Gly CoA DMglyc H2O Formol Sar H2O Formol Lipoate 8As6SA CO2 Dhlip NH3 5XM-THF Accumulation Gly H2O THFA Ser NAD NADH OH-Pyr Ala Glyoxal Arg GIAA OMP SAH SAMe Creatin Suc-CoA 5-Amnva CoA βine Hcys Met Glycera ATP ADP NAD 3POHPyr NADH Glu SEP AKG H2O Pi NH3 THFA Accumulation Gly 5XM-THF H2O D-Ser Hcys L-Cystt H2O ATP AMP Ppi ATP AMP Ppi H2O 2KBA NH3 SAH SAMe LST LSTS GT GTG PW000018 PW000152 PW000146 PW000054 PW000054 PW000054 PW000148