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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 Accumulation L-Alanine Oxalacetic acid Pyruvic acid Pyruvic acid ATP AMP PPi Oxoglutaric acid L-Glutamic acid ATP HCO3- ADP Pi Accumulation L-Alanine Glyoxylic acid Glycine ATP AMP PPi Pyridoxal 5'-phosphate Pyridoxal 5'-phosphate Zinc (II) ion tRNA(Ala) L-Alanyl- tRNA(Ala) tRNA(Ala) L-Alanyl- tRNA(Ala) Selenoamino Acid Metabolism Citric Acid Cycle Biotin Manganese Zinc (II) ion Mitochondria Intracellular Space Intermembrane space Mitochondrial Matrix Cytosol
Mitochondria MPC1 AARS GPT PC AGXT AARS2 Accumulation 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 Accumulation L-Alanine 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 Accumulation Ala Oaa Pyr Pyr ATP AMP PPi AKG Glu ATP HCO3- ADP Pi Accumulation Ala Glyoxal Gly ATP AMP PPi Pyr-5'P Pyr-5'P Zinc TRNAALA LATA TRNAALA LATA PW000007 PW000005 Biotin Mn2+ Zinc Mitochondria Intracellular Space Intermembrane space Mitochondrial Matrix Cytosol
Mitochondria MPC1 AARS GPT PC AGXT AARS2 Accumulation Ala Oaa Pyr Pyr ATP AMP Ppi