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Mitochondrial pyruvate carrier 1 Monocarboxylate transporter 4 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 Glyoxylic acid ATP AMP PPi Oxoglutaric acid L-Glutamic acid ATP HCO3- ADP Pi L-Alanine Glycine ATP AMP PPi Pyridoxal 5'-phosphate Pyridoxal 5'-phosphate Zinc (II) ion Glyoxylic acid Monocarboxylate transporter 4 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 Symptoms include hematuria, myocarditis, nephrocalcinosis, peripheral neuropathy, and renal failure.
Mitochondria MPC1 SLC16A3 AARS GPT PC AGXT AARS2 L-Alanine Oxalacetic acid Pyruvic acid Pyruvic acid Glyoxylic acid Adenosine triphosphate Adenosine monophosphate Pyrophosphate Oxoglutaric acid L-Glutamic acid Adenosine triphosphate Hydrogen carbonate Adenosine diphosphate Phosphate L-Alanine Glycine Adenosine triphosphate Adenosine monophosphate Pyrophosphate Glyoxylic acid SLC16A3 tRNA(Ala) L-Alanyl- tRNA(Ala) tRNA(Ala) L-Alanyl- tRNA(Ala) Selenoamino Acid Metabolism Citric Acid Cycle