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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
PP
i
Oxoglutaric acid
L-Glutamic acid
ATP
HCO
3
-
ADP
P
i
Accumulation
L-Alanine
Glyoxylic acid
Glycine
ATP
AMP
PP
i
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
PP
i
AKG
Glu
ATP
HCO
3
-
ADP
P
i
Accumulation
Ala
Glyoxal
Gly
ATP
AMP
PP
i
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