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Mitochondrial pyruvate carrier 1 Neutral amino acid transporter A Unknown Monocarboxylate transporter 11 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 L-Alanine Oxoglutaric acid Oxoglutaric acid Pyruvic acid ATP AMP PPi L-Glutamic acid ATP HCO3- ADP Pi 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 Cytosol Biotin Manganese Zinc (II) ion Mitochondria Intracellular Space Intermembrane space Mitochondrial Matrix Symptoms include ataxia, lactic acidosis, mental retardation, metabolic acidosis, siezures, and dyspnea.
Mitochondria MPC1 SLC1A4 Unknown SLC16A11 AARS GPT PC AGXT AARS2 L-Alanine Oxalacetic acid Pyruvic acid Pyruvic acid L-Alanine Oxoglutaric acid Oxoglutaric acid Pyruvic acid Adenosine triphosphate Adenosine monophosphate Pyrophosphate L-Glutamic acid Adenosine triphosphate Hydrogen carbonate Adenosine diphosphate Phosphate 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