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Cystinosin
Aspartate
aminotransferase,
cytoplasmic
3-
mercaptopyruvate
sulfurtransferase
L-lactate
dehydrogenase
A-like 6A
Cysteine
dioxygenase
type 1
Cystathionine
gamma-lyase
Aspartate
aminotransferase,
cytoplasmic
Glutamate--
cysteine ligase
Cysteine--tRNA
ligase,
cytoplasmic
L-Glutamic acid
γ-Glutamylcysteine
3-Sulfinoalanine
Pyruvic acid
L-Cysteine
L-Cysteine
Oxoglutaric acid
3-Sulfinylpyruvic acid
L-Glutamic acid
H
2
O
Sulfite
3-Mercaptopyruvic acid
Cyanide
Thiocyanate
3-Mercaptolactic acid
NAD
NADH
O
2
H
2
O
Hydrogen sulfide
NH
3
Oxoglutaric acid
L-Glutamic acid
ATP
ADP
P
i
ATP
AMP
PP
i
Fe2+
Pyridoxal
5'-phosphate
tRNA(Cys)
L-
Cysteinyl-
tRNA(Cys)
Glutamate
Metabolism
Glutamate
Metabolism
Taurine and
Hypotaurine
Metabolism
Sulfate/Sulfite
Metabolism
Pyruvate
Metabolism
Methionine
Metabolism
Glycine and
Serine
Metabolism
Pantothenate
and CoA
Biosynthesis
Pyridoxal
5'-phosphate
Pyridoxal
5'-phosphate
Zinc (II) ion
Lysosome
Cytosol
Extracellular Space
Low IQ, grand mal seizures,
flattened nasal bridge, and
an excessively arched palate
Lysosome
CTNS
GOT1
MPST
LDHAL6A
CDO1
CTH
GOT1
GCLC
GCLM
CARS
L-Glutamic acid
γ-
Glutamylcysteine
3-
Sulfinoalanine
Pyruvic acid
L-Cysteine
L-Cysteine
Oxoglutaric
acid
3-
Sulfinylpyruvic
acid
L-Glutamic acid
Water
Sulfite
3-
Mercaptopyruvic
acid
Cyanide
Thiocyanate
3-
Mercaptolactic
acid
NAD
NADH
Oxygen
Water
Hydrogen
sulfide
Ammonia
Oxoglutaric
acid
L-Glutamic acid
Adenosine
triphosphate
Adenosine
diphosphate
Phosphoric acid
Adenosine
triphosphate
Adenosine
monophosphate
Pyrophosphate
tRNA(Cys)
L-
Cysteinyl-
tRNA(Cys)
Glutamate
Metabolism
Glutamate
Metabolism
Taurine and
Hypotaurine
Metabolism
Sulfate/Sulfite
Metabolism
Pyruvate
Metabolism
Methionine
Metabolism
Glycine and
Serine
Metabolism
Pantothenate
and CoA
Biosynthesis