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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
PP
i
Oxoglutaric acid
L-Glutamic acid
ATP
HCO
3
-
ADP
P
i
L-Alanine
Glycine
ATP
AMP
PP
i
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