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Amine oxidase [flavin- containing] A Aldehyde dehydrogenase, mitochondrial 5- aminolevulinate synthase, erythroid- specific, mitochondrial Dimethylglycine dehydrogenase, mitochondrial Sarcosine dehydrogenase, mitochondrial Glycine decarboxylase Aminomethyltransferase Serine hydroxymethyltransferase Dihydrolipoyl dehydrogenase, mitochondrial Serine-- pyruvate aminotransferase, mitochondrial Serine-- pyruvate aminotransferase, mitochondrial Glycine amidinotransferase, mitochondrial Guanidinoacetate N- methyltransferase 5- aminolevulinate synthase, nonspecific, mitochondrial Betaine-- homocysteine S- methyltransferase 1 Glycine N- methyltransferase Glycerate kinase D-3- phosphoglycerate dehydrogenase Phosphoserine aminotransferase Phosphoserine phosphatase L-serine dehydratase/L- threonine deaminase Serine hydroxymethyltransferase Serine racemase Cystathionine beta-synthase Cystathionine gamma-lyase Serine--tRNA ligase, cytoplasmic Glycine--tRNA ligase 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 S-Adenosylhomocysteine S-Adenosylmethionine Accumulation Glycine 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 2-Ketobutyric acid NH3 ATP Adenosine monophosphate PPi ATP AMP PPi FAD Pyridoxal 5'-phosphate FAD FAD Pyridoxal 5'-phosphate Pyridoxal 5'-phosphate FAD Pyridoxal 5'-phosphate Pyridoxal 5'-phosphate Pyridoxal 5'-phosphate Zinc (II) ion Pyridoxal 5'-phosphate Magnesium Pyridoxal 5'-phosphate Pyridoxal 5'-phosphate 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 Intracellular Space Mitochondria Outer mitochondria membrane Inner mitochondria membrane Mitochondria matrix
Mitochondria Maoa Aldh2 Alas2 Dmgdh Sardh Gldc Amt Shmt2 Dld Agxt Agxt Gatm Gamt Alas1 Bhmt Gnmt Glyctk Phgdh Unknown Psph Sds Shmt1 Srr Cbs Cth Sars Gars 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 S- Adenosylhomocysteine S- Adenosylmethionine Accumulation Glycine 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 2-Ketobutyric acid Ammonia Adenosine triphosphate Adenosine monophosphate Pyrophosphate Adenosine triphosphate Adenosine monophosphate Pyrophosphate 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 Alas2 Dmgdh Sardh Gldc Amt Shmt2 Dld Agxt Agxt Gatm Gamt Alas1 Bhmt Gnmt Glyctk Phgdh Psph Sds Shmt1 Srr Cbs Cth Sars Gars 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 SAH SAMe Accumulation Gly Glycera ATP ADP NAD 3POHPyr NADH Glu SEP AKG H2O Pi NH3 THFA Accumulation Gly 5XM-THF H2O D-Ser Hcys L-Cystt H2O 2KBA NH3 ATP AMP PPi ATP AMP PPi FAD Pyr-5'P FAD FAD Pyr-5'P Pyr-5'P FAD Pyr-5'P Pyr-5'P Pyr-5'P Zinc Pyr-5'P Mg2+ Pyr-5'P Pyr-5'P Pyr-5'P Pyr-5'P Pyr-5'P LST LSTS GT GTG PW000018 PW000152 PW000146 PW000054 PW000054 PW000054 PW000148 Intracellular Space Mitochondria Outer mitochondria membrane Inner mitochondria membrane Mitochondria matrix
Mitochondria Maoa Aldh2 Alas2 Dmgdh Sardh Gldc Amt Shmt2 Dld Agxt Agxt Gatm Gamt Alas1 Bhmt Gnmt Glyctk Phgdh Psph Sds Shmt1 Srr Cbs Cth Sars Gars 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 SAH SAMe Accumulation Gly Glycera ATP ADP NAD 3POHPyr NADH Glu SEP AKG H2O Pi NH3 THFA Accumulation Gly 5XM-THF H2O D-Ser Hcys L-Cystt H2O 2KBA NH3 ATP AMP Ppi ATP AMP Ppi LST LSTS GT GTG PW000018 PW000152 PW000146 PW000054 PW000054 PW000054 PW000148