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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
1A
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
Amine oxidase
[flavin-
containing] A
Amine oxidase
[flavin-
containing] A
3-
hydroxyanthranilate
3,4-dioxygenase
Catechol O-
methyltransferase
Aldehyde
dehydrogenase,
dimeric
NADP-preferring
L-Tyrosine
Oxoglutaric acid
4-Hydroxyphenylpyruvic acid
L-Glutamic acid
L-Aspartic acid
Oxalacetic acid
O
2
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
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
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
NADPH
Guaiacol
Pyrocatechol
Guaiacol
Vanylglycol
Pyrocatechol
Guaiacol
Pyrocatechol
Guaiacol
3-Methoxytyramine
Pyrocatechol
H
2
O
O
2
3,4-
Dihydroxyphenylacetaldehyde
NH
3
H
2
O
2
H
2
O
O
2
Homovanillin
H
2
O
2
NH
3
NAD
H
2
O
3,4-Dihydroxybenzeneacetic
acid
NADH
H
+
Guaiacol
Homovanillic acid
Pyrocatechol
NAD
H
2
O
NADH
H
+
Pyridoxal
5'-phosphate
Pyridoxal
5'-phosphate
Pyridoxal
5'-phosphate
Pyrroloquinoline
quinone
Copper
Copper
Copper
Copper
FAD
FAD
Zinc
Topaquinone
Copper
Magnesium
Magnesium
Magnesium
Magnesium
Magnesium
FAD
FAD
Fe2+
Magnesium
Epinephrine
Vanillylmandelic acid
Phenylalanine
and Tyrosine
Metabolism
Thyroid Hormone
Synthesis
Fe3+
Magnesium
Copper
Pyridoxal
5'-phosphate
Zinc
Copper
FAD
FAD
Iron
Copper
Calcium
FAD
Calcium
Cytosol
Extracellular space
Melanosome
Melanosome
Melanosome
Nasal stuffiness
Orthostatic hypotension
Ptosis and a neonatal
history of delayed eye
opening.
TAT
GOT1
HPD
GSTZ1
FAH
DDC
ALDH3A1
TYR
DBH
DDC
TYR
TYR
TYR
DCT
TYR
PNMT
MAOA
MAOA
MAOA
MAOA
ALDH3A1
ADH1A
HGD
MIF
ABP1
MAOA
TYR
COMT
ALDH3A1
COMT
COMT
COMT
COMT
MAOA
MAOA
HAAO
COMT
ALDH3A1
L-Tyrosine
Oxoglutaric
acid
4-
Hydroxyphenylpyruvic
acid
L-Glutamic acid
L-Aspartic acid
Oxalacetic acid
Oxygen
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
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
Oxygen
2-Hydroxy-3-
(4-
hydroxyphenyl)propenoic
acid
Water
Oxygen
Hydrogen
peroxide
Ammonia
Water
Oxygen
Hydrogen
peroxide
Ammonia
Oxygen
Water
Guaiacol
Pyrocatechol
NADP
Water
NADPH
Guaiacol
Pyrocatechol
Guaiacol
Vanylglycol
Pyrocatechol
Guaiacol
Pyrocatechol
Guaiacol
3-
Methoxytyramine
Pyrocatechol
Water
Oxygen
3,4-
Dihydroxyphenylacetaldehyde
Ammonia
Hydrogen
peroxide
Water
Oxygen
Homovanillin
Hydrogen
peroxide
Ammonia
NAD
Water
3,4-
Dihydroxybenzeneacetic
acid
NADH
Hydrogen Ion
Guaiacol
Homovanillic
acid
Pyrocatechol
NAD
Water
NADH
Hydrogen Ion
Epinephrine
Vanillylmandelic
acid
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
ADH1A
HGD
MIF
ABP1
MAOA
TYR
COMT
ALDH3A1
COMT
COMT
COMT
COMT
MAOA
MAOA
HAAO
COMT
ALDH3A1
Tyr
AKG
4HPPA
Glu
Asp
Oaa
O
2
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
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
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
NADPH
Guajol
Catecho
Guajol
Vanygly
Catecho
Guajol
Catecho
Guajol
3-Motyr
Catecho
H
2
O
O
2
3,4-HPA
NH
3
H
2
O
2
H
2
O
O
2
Homovan
H
2
O
2
NH
3
NAD
H
2
O
34DHBzA
NADH
H
+
Guajol
HVA
Catecho
NAD
H
2
O
NADH
H
+
Pyr-5'P
Pyr-5'P
Pyr-5'P
Pqq
Cu
Cu
Cu
Cu
FAD
FAD
Zinc
TopQne
Cu
Mg2+
Mg2+
Mg2+
Mg2+
Mg2+
FAD
FAD
Fe2+
Mg2+
Eppy
VMA
PW000042
PW000693
Fe3+
Mg2+
Cu
Pyr-5'P
Zinc
Cu
FAD
FAD
Iron
Cu
Ca2+
FAD
Ca2+
Cytosol
Extracellular space
Melanosome
Melanosome
Melanosome
Nasal stuffiness
Orthostatic hypotension
Ptosis and a neonatal
history of delayed eye
opening.