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Tyrosine
aminotransferase
Aspartate
aminotransferase,
cytoplasmic
4-
hydroxyphenylpyruvate
dioxygenase
Maleylacetoacetate
isomerase
Fumarylacetoacetase
Aromatic-L-
amino-acid
decarboxylase
Aldehyde
dehydrogenase,
dimeric
NADP-preferring
Tyrosinase
Dopamine beta-
hydroxylase
Aromatic-L-
amino-acid
decarboxylase
Tyrosinase
Tyrosinase
Tyrosinase
L-dopachrome
tautomerase
Tyrosinase
Phenylethanolamine
N-
methyltransferase
Amine oxidase
[flavin-
containing] A
Amine oxidase
[flavin-
containing] A
Amine oxidase
[flavin-
containing] A
Amine oxidase
[flavin-
containing] A
Aldehyde
dehydrogenase,
dimeric
NADP-preferring
Alcohol
dehydrogenase 1
Amine oxidase
[flavin-
containing] A
Amine oxidase
[flavin-
containing] A
3-
hydroxyanthranilate
3,4-dioxygenase
Aldehyde
dehydrogenase,
dimeric
NADP-preferring
Homogentisate
1,2-dioxygenase
Macrophage
migration
inhibitory
factor
Amiloride-
sensitive amine
oxidase
[copper-
containing]
Amine oxidase
[flavin-
containing] A
Tyrosinase
Catechol O-
methyltransferase
Aldehyde
dehydrogenase,
dimeric
NADP-preferring
Catechol O-
methyltransferase
Catechol O-
methyltransferase
Catechol O-
methyltransferase
Catechol O-
methyltransferase
Catechol O-
methyltransferase
L-Tyrosine
Oxoglutaric
acid
4-
Hydroxyphenylpyruvic
acid
L-Glutamic acid
L-Aspartic acid
Oxalacetic acid
O
2
Accumulation
Homogentisic
acid
CO
2
Maleylacetoacetic
acid
4-
Fumarylacetoacetic
acid
H
2
O
Acetoacetic
acid
Fumaric acid
Tyramine
CO
2
Homovanillin
NADP
H
2
O
p-
Hydroxyphenylacetic
acid
NADPH
O
2
Dopamine
H
2
O
Ascorbic acid
O
2
Norepinephrine
Dehydroascorbic
acid
H
2
O
L-Dopa
Tetrahydrobiopterin
4a-
Hydroxytetrahydrobiopterin
O
2
Dopaquinone
H
2
O
Leucodopachrome
L-Dopachrome
O
2
5,6-
Dihydroxyindole
H
2
O
O
2
Indole-5,6-
quinone
H
2
O
Melanin
5,6-
Dihydroxyindole-
2-carboxylic
acid
O
2
H
2
O
S-
Adenosylmethionine
S-
Adenosylhomocysteine
Epinephrine
H
2
O
O
2
3,4-
Dihydroxymandelaldehyde
NH
3
H
2
O
2
H
2
O
O
2
Methylamine
H
2
O
2
Normetanephrine
H
2
O
O
2
3-Methoxy-4-
hydroxyphenylglycolaldehyde
NH
3
H
2
O
2
Metanephrine
H
2
O
O
2
NH
3
H
2
O
2
NAD
H
2
O
3,4-
Dihydroxymandelic
acid
NADH
H
+
NAD
NADH
3,4-
Dihydroxyphenylglycol
Dopamine
H
2
O
O
2
3,4-
Dihydroxyphenylacetaldehyde
NH
3
H
2
O
2
3-
Methoxytyramine
H
2
O
O
2
Homovanillin
H
2
O
2
NH
3
NAD
H
2
O
3,4-
Dihydroxybenzeneacetic
acid
NADH
H
+
NAD
H
2
O
Homovanillic
acid
NADH
H
+
O
2
2-Hydroxy-3-
(4-
hydroxyphenyl)propenoic
acid
H
2
O
O
2
H
2
O
2
NH
3
H
2
O
O
2
H
2
O
2
NH
3
O
2
H
2
O
Guaiacol
Pyrocatechol
NADP
H
2
O
Vanillylmandelic
acid
NADPH
Guaiacol
Pyrocatechol
Guaiacol
Vanylglycol
Pyrocatechol
Guaiacol
Pyrocatechol
Guaiacol
Pyrocatechol
Guaiacol
Pyrocatechol
Pyridoxal
5'-phosphate
Pyridoxal
5'-phosphate
Fe3+
Calcium
Magnesium
Pyridoxal
5'-phosphate
Copper
Pyrroloquinoline
quinone
Copper
Pyridoxal
5'-phosphate
Copper
Copper
Copper
Zinc
Copper
FAD
FAD
FAD
FAD
Zinc
FAD
FAD
Iron
Copper
Calcium
Topaquinone
FAD
Copper
Magnesium
Magnesium
Magnesium
Magnesium
Magnesium
Magnesium
Phenylalanine
and Tyrosine
Metabolism
Thyroid Hormone
Synthesis
Intracellular space
Extracellular space
Tat
Got1
Hpd
Gstz1
Fah
Ddc
Aldh3a1
Tyr
Dbh
Ddc
Tyr
Tyr
Tyr
Dct
Tyr
Pnmt
Maoa
Maoa
Maoa
Maoa
Aldh3a1
Adh1
Maoa
Maoa
Haao
Aldh3a1
Hgd
Mif
Aoc1
Maoa
Tyr
Comt
Aldh3a1
Comt
Comt
Comt
Comt
Comt
L-Tyrosine
Oxoglutaric
acid
4-
Hydroxyphenylpyruvic
acid
L-Glutamic acid
L-Aspartic acid
Oxalacetic acid
Oxygen
Accumulation
Homogentisic
acid
Carbon dioxide
Maleylacetoacetic
acid
4-
Fumarylacetoacetic
acid
Water
Acetoacetic
acid
Fumaric acid
Tyramine
Carbon dioxide
Homovanillin
NADP
Water
p-
Hydroxyphenylacetic
acid
NADPH
Oxygen
Dopamine
Water
Ascorbic acid
Oxygen
Norepinephrine
Dehydroascorbic
acid
Water
L-Dopa
Tetrahydrobiopterin
4a-
Hydroxytetrahydrobiopterin
Oxygen
Dopaquinone
Water
Leucodopachrome
L-Dopachrome
Oxygen
5,6-
Dihydroxyindole
Water
Oxygen
Indole-5,6-
quinone
Water
Melanin
5,6-
Dihydroxyindole-
2-carboxylic
acid
Oxygen
Water
S-
Adenosylmethionine
S-
Adenosylhomocysteine
Epinephrine
Water
Oxygen
3,4-
Dihydroxymandelaldehyde
Ammonia
Hydrogen
peroxide
Water
Oxygen
Methylamine
Hydrogen
peroxide
Normetanephrine
Water
Oxygen
3-Methoxy-4-
hydroxyphenylglycolaldehyde
Ammonia
Hydrogen
peroxide
Metanephrine
Water
Oxygen
Ammonia
Hydrogen
peroxide
NAD
Water
3,4-
Dihydroxymandelic
acid
NADH
Hydrogen Ion
NAD
NADH
3,4-
Dihydroxyphenylglycol
Dopamine
Water
Oxygen
3,4-
Dihydroxyphenylacetaldehyde
Ammonia
Hydrogen
peroxide
3-
Methoxytyramine
Water
Oxygen
Homovanillin
Hydrogen
peroxide
Ammonia
NAD
Water
3,4-
Dihydroxybenzeneacetic
acid
NADH
Hydrogen Ion
NAD
Water
Homovanillic
acid
NADH
Hydrogen Ion
Oxygen
2-Hydroxy-3-
(4-
hydroxyphenyl)propenoic
acid
Water
Oxygen
Hydrogen
peroxide
Ammonia
Water
Oxygen
Hydrogen
peroxide
Ammonia
Oxygen
Water
Guaiacol
Pyrocatechol
NADP
Water
Vanillylmandelic
acid
NADPH
Guaiacol
Pyrocatechol
Guaiacol
Vanylglycol
Pyrocatechol
Guaiacol
Pyrocatechol
Guaiacol
Pyrocatechol
Guaiacol
Pyrocatechol
Phenylalanine
and Tyrosine
Metabolism
Thyroid Hormone
Synthesis
Tat
Got1
Hpd
Gstz1
Fah
Ddc
Aldh3a1
Tyr
Dbh
Ddc
Tyr
Tyr
Tyr
Dct
Tyr
Pnmt
Maoa
Maoa
Maoa
Maoa
Aldh3a1
Adh1
Maoa
Maoa
Haao
Aldh3a1
Hgd
Mif
Aoc1
Maoa
Tyr
Comt
Aldh3a1
Comt
Comt
Comt
Comt
Comt
Tyr
AKG
4HPPA
Glu
Asp
Oaa
O
2
Accumulation
HMGNCA
CO
2
MalaceA
Fumaryl
H
2
O
LIN
Fumarat
Tyramin
CO
2
Homovan
NADP
H
2
O
p-Hpaa
NADPH
O
2
LDP
H
2
O
VitC
O
2
Norpp
DHAA
H
2
O
L-Dopa
BH4
4aHtHbp
O
2
Dopqone
H
2
O
Leucpch
L-Dopac
O
2
56-DHD
H
2
O
O
2
ind56q
H
2
O
Melanin
DHD-2CA
O
2
H
2
O
SAMe
SAH
Eppy
H
2
O
O
2
34DIHY
NH
3
H
2
O
2
H
2
O
O
2
Methyla
H
2
O
2
Normeta
H
2
O
O
2
4H3M
NH
3
H
2
O
2
Meta-np
H
2
O
O
2
NH
3
H
2
O
2
NAD
H
2
O
34-DHMA
NADH
H
+
NAD
NADH
DHPG
LDP
H
2
O
O
2
3,4-HPA
NH
3
H
2
O
2
3-Motyr
H
2
O
O
2
Homovan
H
2
O
2
NH
3
NAD
H
2
O
34DHBzA
NADH
H
+
NAD
H
2
O
HVA
NADH
H
+
O
2
4AD
H
2
O
O
2
H
2
O
2
NH
3
H
2
O
O
2
H
2
O
2
NH
3
O
2
H
2
O
Guajol
Catecho
NADP
H
2
O
VMA
NADPH
Guajol
Catecho
Guajol
Vanygly
Catecho
Guajol
Catecho
Guajol
Catecho
Guajol
Catecho
Pyr-5'P
Pyr-5'P
Fe3+
Ca2+
Mg2+
Pyr-5'P
Cu
Pqq
Cu
Pyr-5'P
Cu
Cu
Cu
Zinc
Cu
FAD
FAD
FAD
FAD
Zinc
FAD
FAD
Iron
Cu
Ca2+
TopQne
FAD
Cu
Mg2+
Mg2+
Mg2+
Mg2+
Mg2+
Mg2+
PW000042
PW000693
Intracellular space
Extracellular space
Tat
Got1
Hpd
Gstz1
Fah
Ddc
Aldh3a1
Tyr
Dbh
Ddc
Tyr
Tyr
Tyr
Dct
Tyr
Pnmt
Maoa
Maoa
Maoa
Maoa
Aldh3a1
Adh1
Maoa
Maoa
Haao
Aldh3a1
Hgd
Mif
Aoc1
Maoa
Tyr
Comt
Aldh3a1
Comt
Comt
Comt
Comt
Comt
Tyr
AKG
4HPPA
Glu
Asp
Oaa
O2
Accumulation
HMGNCA
CO2
MalaceA
Fumaryl
H2O
LIN
Fumarat
Tyramin
CO2
Homovan
NADP
H2O
p-Hpaa
NADPH
O2
LDP
H2O
VitC
O2
Norpp
DHAA
H2O
L-Dopa
BH4
4aHtHbp
O2
Dopqone
H2O
Leucpch
L-Dopac
O2
56-DHD
H2O
O2
ind56q
H2O
Melanin
DHD-2CA
O2
H2O
SAMe
SAH
Eppy
H2O
O2
34DIHY
NH3
H2O2
H2O
O2
Methyla
H2O2
Normeta
H2O
O2
4H3M
NH3
H2O2
Meta-np
H2O
O2
NH3
H2O2
NAD
H2O
34-DHMA
NADH
H+
NAD
NADH
DHPG
LDP
H2O
O2
3,4-HPA
NH3
H2O2
3-Motyr
H2O
O2
Homovan
H2O2
NH3
NAD
H2O
34DHBzA
NADH
H+
NAD
H2O
HVA
NADH
H+
O2
4AD
H2O
O2
H2O2
NH3
H2O
O2
H2O2
NH3
O2
H2O
Guajol
Catecho
NADP
H2O
VMA
NADPH
Guajol
Catecho
Guajol
Vanygly
Catecho
Guajol
Catecho
Guajol
Catecho
Guajol
Catecho
PW000042
PW000693