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Mitochondrial pyruvate carrier 1 Alanine--tRNA ligase, cytoplasmic Alanine aminotransferase 1 Pyruvate carboxylase, mitochondrial Serine-- pyruvate aminotransferase Alanine--tRNA ligase, mitochondrial L-Alanine Oxalacetic acid Pyruvic acid Pyruvic acid ATP AMP PPi Oxoglutaric acid L-Glutamic acid ATP HCO3- ADP Pi L-Alanine Accumulation Glyoxylic acid Glycine ATP AMP PPi Zinc (II) ion Pyridoxal 5'-phosphate Biotin Manganese Pyridoxal 5'-phosphate Zinc (II) ion tRNA(Ala) L-Alanyl- tRNA(Ala) tRNA(Ala) L-Alanyl- tRNA(Ala) Selenoamino Acid Metabolism Citric Acid Cycle Mitochondria Intracellular Space Intermembrane space Matrix
Mitochondria MPC1 AARS GPT PC AGXT AARS2 L-Alanine Oxalacetic acid Pyruvic acid Pyruvic acid Adenosine triphosphate Adenosine monophosphate Pyrophosphate Oxoglutaric acid L-Glutamic acid Adenosine triphosphate Hydrogen carbonate Adenosine diphosphate Phosphate L-Alanine Accumulation Glyoxylic acid Glycine Adenosine triphosphate Adenosine monophosphate Pyrophosphate tRNA(Ala) L-Alanyl- tRNA(Ala) tRNA(Ala) L-Alanyl- tRNA(Ala) Selenoamino Acid Metabolism Citric Acid Cycle
MPC1 AARS GPT PC AGXT AARS2 Ala Oaa Pyr Pyr ATP AMP PPi AKG Glu ATP HCO3- ADP Pi Ala Accumulation Glyoxal Gly ATP AMP PPi Zinc Pyr-5'P Biotin Mn2+ Pyr-5'P Zinc TRNAALA LATA TRNAALA LATA PW000007 PW000005 Mitochondria Intracellular Space Intermembrane space Matrix
Mitochondria MPC1 AARS GPT PC AGXT AARS2 Ala Oaa Pyr Pyr ATP AMP Ppi AKG Glu ATP HCO3 ADP Pi Ala Accumulation Glyoxal Gly ATP AMP Ppi TRNAALA LATA TRNAALA LATA PW000007 PW000005