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Pathways of Human Metabolism

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A
B
Cholesterol synthesis
Glycogen and galactose metabolism
acetyl-CoA x2
1
2
3
4
5
6
8
9
CoA
acetoacetyl-CoA
acetyl-CoA
HMG-CoA synthase
CoA
glycogen (n)
6
3
4
5
α-1,6 bond
Steroid hormone synthesis
Zona glomerulosa
2 NADPH
HMG-CoA reductase
CoA
UDP-glucuronate
Cholesterol
desmolase
17α-hydroxylase
pregnenolone
3 ADP
CO2
isopentenyl pyrophosphate (IPP)
(C5) x3
(multiple
steps)
8 NADP
(multiple
steps)
Glycerol-3-phosphate
acyltransferase
(GPAT)
NADPH
protein
modification
NADP+
glycerol
ATP ADP
Triacylglycerol
synthetase
complex
B12
folate
cobalamin
4'
2'
3'
4'
glucose-6-phosphate
glutamine
PPi
Glutamine: F6P
aminotransferase
ATP
Phosphofructokinase-1
ADP
B6
Coenzyme Q (oxidized)
AMP
ATP
phospholipids
1,2-diacylglycerol
Long-chain
fatty
acyl-CoA
synthetase
β-ketoacyl-ACP
fatty acyl-CoA
β-hydroxyacyl-ACP
dehydratase
palmitate (C16)
ADP
Acetyl-CoA
carboxylase
dihydroxyacetone
phosphate (DHAP)
HCO3
fatty acids
≤ C12
fatty acids
> C24 to peroxisomes
fatty acids
> C14 and < C22
Pi
Heme
metabolism
4
aspartate
tricarboxylate
transporter
Fatty acid oxidation
PPi
NAD
+
NADP+ NADPH
malate
fatty acyl- CoA
fatty acids ≤ C12
Multiple steps
Multiple cycles
Crosses membrane
aspartate
Acyl-CoA
Synthetase
Acyl-CoA
dehydrogenases
Hydratase
CoA
Acetyl-CoA
synthetase
5
PPi
Enzyme cofactor:
α-ketoacid dehydrogenases and
transketolase
Precursor:
FAD/FADH2
Hydroxyacyl-CoA
dehydrogenases
AMP
HMG-CoA synthase (in liver)
CoA
Thiolase
Enzyme cofactor:
transaminases and other reactions
involving amino acids
Heme synthesis
Enzyme cofactor: carboxylases
glycine
Single-carbon carrier
succinyl-CoA
FAD
NADH
NAD+
CO2
δ-aminolevulinic
acid synthase B6
VERSION 10.18
Copyright 2017 Stanford School of Medicine. All rights reserved.
This work is licensed under a Creative Commons AttributionNonCommercial-NoDerivatives 4.0 International License.
lactate
Methylmalonyl-CoA mutase
NAD
Citric acid cycle (tricarboxylic acid cycle)
Pyruvate B1
dehydrogenase
CoA Branched-chain
ketoacid B1
CO2 dehydrogenase
(BCKAD)
NAD+
CoA
succinyl-CoA
succinate
acetoacetate
acetoacetyl-CoA
Succinyl-CoA:acetoacetate
β-hydroxybutyrate
transferase (SCOT)
dehydrogenase
(in extrahepatic tissues)
Citrate
synthase
oxaloacetate
CoA
H2O
Propionyl-CoA carboxylase
B7
H2O
NH4
Fumarase
CoA
α-ketoglutarate
B
dehydrogenase 1
Thiolase
acetyl-CoA x2
3 H2O2
H2O
3 O2
Protoporphyrinogen
IX oxidase
Succinyl-CoA
synthetase GDP
many
amino Transaminases
acids
NADH
hydroxymethylbilane
uroporphyrinogen III
Uroporphyrinogen III
Uroporphyrinogen
4 NH4+
synthase
decarboxylase
D
Succinic semialdehyde
dehydrogenase
CoA
Glutamate
decarboxylase
2 H2O
Coproporphyrinogen oxidase
-
+
+
4H
+
I
-
+
-
+
-
+
-
-
+
-
+
-
+
+
2 e-
FAD
- - -
-
-
-
-
-
-
+
+
+
+
+
+
+
+
+
-
-
2e
CoQ
NADH
dehydrogenase
(Complex I)
-
GABA
aminotransferase
+
+
-
-
-III -
-
-
-
-- -
2e
-
-
-
-
-
2e
-
-
-
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
ADP
Pi
-
IV
+
+
-
NADPH NADP+
+
Cytochrome c
oxidase
(Complex IV) 2 H+
biliverdin
+
-
+
-
+
-
-
+
-
+
ATP synthase
(Complex V)
F
UDP x2
bilirubin
Biliverdin reductase
3 O2 Fe 3+ CO
Heme oxygenase
UDP-glucuronate x2
Pi
Ornithine
transcarbamoylase (OTC)
ornithine
H2O
Nucleotidase
Pi
AMP
deaminase
+
3 H+
+
-
+
-
-
phosphate
transporter
+
+
Pi
-
-
-
-
-
H2O
adenine nucleotide
transporter
+
+
+
ADP
+
NH4+
+
Nucleotidase
(deoxy)
ribose-1-P
(deoxy)
adenosine
Adenosine
deaminase
H2O
Guanine
deaminase
conjugated bilirubin
UDP-glucuronyltransferase
G
B6
sulfite (SO3 )
Sulfite
oxidase
taurine
bile salts
Xanthine oxidase
feces
urine
bile
NH4
UTP
ATP
CTP synthetase
ADP + Pi
aminoiminidazole carboxamide ribonucleotide
(AICAR)
N10-formyl-THF
AICAR transformylase
urea
IMP cyclohydrolase
arginine
NH4+
H2O
Nucleotidase
adenylosuccinate
pyrroline-5-carboxylate
PRPP
Adenine
phosphoribosyltransferase
AMP
(deoxy)
inosine
Thymidine
phosphorylase
Pi
(deoxy)
ribose-1-P
NADPH
NADP+
β-ureidopropionate
CO2
ATP
adenine
guanine
PRPP
ATP
GMP synthetase
glutamate
HGPRT
PRPP
AMP + PPi
GMP
PPi
ATP
ADP
PRPP synthetase
ATP
ribose-5-P
GTP
GMP kinase
ADP
GDP
Phosphopentomutase
Nucleoside
diphosphate
kinase
glutamine
AMP
ADP
UDP
Pi
ATP
Kinase
ADP
deoxy
ribose-1-P
dCDP
ATP
Kinase
ADP
ribose-1-P
uracil
Pi
Uridine phosphorylase
GDP
dATP
NH4
β-alanine
dCTP
dGTP
Phosphatase
NADP+
H2O
Pi
ATP
uridine
ADP
5
Nucleoside
diphosphate
kinase
thioredoxin
(reduced)
β-ureidoisobutyrate
NH4
(spontaneous)
dUTP
pyrophosphatase
Thioredoxin
reductase
NADH, FADH2
dUTP
ATP ADP
adenosine
NADPH
Thymidylate
synthase
(TS)
dUMP
adenosine 5’-monophosphate (AMP)
dTMP
10
SHMT
uridine
dTDP
Kinase
dihydrofolate
ATP
DHFR
ADP
glycine
Uridine-cytidine kinase
cytidine 5’-monophosphate (CMP)
uridine 5’-monophosphate (UMP)
ATP ADP
Kinase
NADP+
deoxycytidine
Deoxycytidine kinase
deoxycytidine 5’-monophosphate (dCMP)
ATP ADP
NADPH
THF
serine
cytidine
ATP ADP
ATP ADP
N ,N methylene-THF
5
Ribonucleotide
reductase
Adenosine kinase
Nucleoside salvage
ATP ADP
PPi
NADP+
thioredoxin
(oxidized)
β-aminoisobutyric
acid
Deoxynucleotide
deaminase
GTP
biopterin
Coenzyme A
ATP
Kinase
ADP
NH4+
dCMP
+
S-adenosyl methionine
NH4+
NADPH
CO2
ATP
dUDP
ATP
Kinase
ADP
dihydrothymine
Ureidopropionase
+
I
H2O
Phosphatase
Pi
CDP
dGDP
Dihydropryimidinase
H2O2
H2O
Phosphatase
Pi
GDP
dADP
thymine
Dihydropyrimidine
dehydrogenase
(DPD)
dihydrouracil
H2O
UTP
(deoxy)
ribose-1-P
uracil
H2O2
CTP
H2O
Phosphatase
Pi
ADP
thymidine
(deoxy)
uridine
Pi
O2
GTP
H2O
Phosphatase
Pi
deoxyTMP
H2O
H2O
Nucleotidase
Pi
Pi
Adenylate
kinase
Hypoxanthine-guanine
phosphoribosyltransferase
(HGPRT)
hypoxanthine
NADP+
Pyrroline-5-carboxylate
reductase
NADP+
GMP reductase
NADPH
XMP
PPi
proline
4
+
Uridine
phosphorylase
H2O
NAD+
IMP dehydrogenase
NADH
fumarate
UMP
(deoxy)
cytidine Pyrimidine
nucleoside
deaminase
H2O
H2O
Base salvage
H2O
pyrroline5-carboxylate
NH4+
Adenylosuccinate
GTP
synthetase
GDP
Pi
AMP
NADPH
CTP
inosine monophosphate
(IMP)
Adenylosuccinate
lyase
(spontaneous)
glutamate
aspartate
asparagine
fumarate
(spontaneous)
H2O
α-ketoglutarate
PPi
NH4+
glutamine
formaminoimidazole carboxamide ribonucleotide
(FAICAR)
ATP
3
Adenylosuccinate lyase
sulfate (SO42-)
NH4+
AMP deaminase
Nucleoside
diphosphate
kinase
NH4
+
uric acid
H
ATP
fumarate
aspartate
ATP
H2O
Pi
Xanthine
oxidase
xanthine
ATP
UDP
H2O
2-
glutamate
Transaminase
NH4+
Asparaginase
H2O
Argininosuccinate
lyase
NADPH
Arginase
ornithine
Pi
(deoxy) IMP
ATP
ADP
Pi
-
glutamate
Deoxyribonucleotides
Nucleotidase
Pi
guanine
ADP
SAICAR synthetase
ADP
pyruvate
β-sulfinylpyruvate
glutamine
Asparagine synthetase
glutamate
ornithine/citrulline
transporter
(deoxy) CMP
Purine nucleoside
phosphorylase
-
B6
oxaloacetate
argininosuccinate
O2
glutamic semialdehyde
(deoxy) AMP
(deoxy) guanosine
Purine
nucleoside
phosphorylase
UMP kinase
aspartate
ADP
NADH
NADP+
H2O
Pi
hypoxanthine
-
hypotaurine
Nitric oxide
synthase
RNA/DNA
H2O
+
TCA
ATP
Pi
Transaminase
Hypotaurine
dehydrogenase
AMP
citrulline
Pi
NADPH NADP+
Pyrroline-5carboxylate synthetase
Pi
urea cycle (regulation)
V
CO2
Argininosuccinate
synthetase
PPi
ATP
NO
glutamate
ATP
ADP + Pi
2 H+
-
H2O
uridine-5'-monophosphate
(UMP)
succinylaminoimidazole carboxamide ribonucleotide
(SAICAR)
α-ketoglutarate
NAD+
ornithine/citrulline
transporter
ATP ADP
O2
3 NADPH 3 NADP+
creatinine
(excreted)
citrulline
Ornithine aminotransferase B6
aspartate
Dehydrogenase
Nucleotidase
3 H+
B6
Cytochrome c
reductase
(Complex III) 4 H+
heme b
E
-
Cysteine sulfinite
decarboxylase
(spontaneous)
H2O
oxaloacetate
(deoxy) GMP
acetate H2O
Hydrolase
γ-aminobutyric
acid (GABA)
Cyt c
Succinate
dehydrogenase
(Complex II)
Heme degradation
coproporphyrinogen III
-
CO2
α-ketoglutarate
/2 O2 + 2H
FADH2
II
- - - - -
Creatine
kinase
polyamines
Nucleotide breakdown
N-acetylglutamate
Oxidative phosphorylation
4 H+
succinate
I
phosphocreatine
succinyl-CoA
OMP decarboxylase
(UMP synthase)
CO2
CO2
ATP
PPi
N-acetylglutamate synthase
acetyl-CoA CoA
1
protoporphyrinogen IX
4 CO2
succinic semialdehyde
NADH
NAD
ADP
ATP
2 ADP
Hydratase
glutamine
many
α-ketoacids
B6
glutamate
NAD+
GTP
+
Pi
B6
NH4+ H2O
ATP
Glutamine synthetase
NH4
cysteine
sulfinate
Nucleases
Glutaminase
NH4+ H2O
nucleotide salvage
Threonine dehydratase
+
threonine
SAM
orotidine-5'-monophosphate (OMP)
aminoimidazole ribonucleotide
(AIR)
Thiolase
acetyl-CoA x2
TCA
O2
Cysteine
dioxygenase
acetoacetyl-CoA
CoA
NADH NAD
Glutamate
dehydrogenase-1
NADH
succinyl-CoA
Pi
4 H+
ornithine
ATP
Pi
H2O
cysteine
Coenzyme A
2 ATP
NADH
glutamate
NAD+
CO2
2 H+
CoA
Methylmalonyl-CoA
mutase B12
+
fumarate
glutathione
Orotate
phosphoribosyltransferase
PPi
ADP
(in mitochondrial matrix)
α-ketobutyrate
HCO3
carbamoyl
phosphate
2
orotate
AIR synthetase
-
Carbamoyl
phosphate
synthetase I
(CPS1)
Dihydroorotate
dehydrogenase
(in mitochondria)
NADH
carboxyaminoimidazole ribonucleotide
(CAIR)
Cystathionine
γ-lyase B6
porphyrins
arginine
crotonyl-CoA
NAD+
succinyl-CoA
α-ketoglutarate
HCO3
NH4
+
β-hydroxybutyryl CoA
methylmalonyl-CoA
Ammonium capture and release
+
-
propionyl-CoA
Propionyl-CoA
carboxylase B7
ADP
AIR carboxylase
Urea cycle
Glutaryl-CoA
dehydrogenase
H2O
NAD+
THF
glutaryl-CoA
odd-chain fatty
acyl-CoA
(last three carbons only)
HCO3-
ADP
acetyl-CoA
acetoacetate
NADH
CO2
ATP
CoA
NAD
Isocitrate
dehydrogenase
NADH
FAD
HMG-CoA lyase
+
B1
dihydroorotate
formiminoglycinamidine ribonucleotide
(FGAM)
cystathionine
B6
CoA
α-ketoadipate B1
dehydrogenase
CO2
FADH2
ATP
β-methylcrotonyl-CoA
carboxylase B7
isocitrate
ADP
NADH
NAD+
isobutyryl-CoA
propionyl-CoA
º-hydroxy-β-methylglutaryl-CoA
(HMG-CoA)
Aconitase
B1
CoA
BCKAD
CO2
acetyl-CoA
β-methylglutaconyl-CoA
hydratase
H2O
NAD+
CH3 (used for methylation)
adenosine
serine
(spontaneous)
Transaminase
Dihydroorotase
PRPP
homocysteine
nucleotides
SAH
N-carbamoyl aspartate
H2O
glutamine
S-adenosylhomocysteine (SAH)
Cystathionine
β-synthase B6
creatine
α-ketoadipate
(multiple steps)
β-methylglutaconyl-CoA
H2O
Aconitase
Methylene-THF
reductase
NADH
α-aminoadipate
glutamate
α-ketoisovalerate
α-methylbutyryl-CoA
HCO3-
ADP
methylmalonyl-CoA
O2
H2O
NADH
β-methylcrotonyl-CoA
citrate
CoA
BCKAD
CO2
NAD+
Isovaleryl-CoA
dehydrogenase
NADH
Ferrochelatase
C
α-ketoisovalerate
α-keto-β-methylvalerate
isovaleryl-CoA
Fe2+
protoporphyrin IX
PBG deaminase
B6
glutamate
α-keto-β-methylvalerate
α-ketoisocaproate
NAD+
B12
NADH
FGAM synthetase
S-adenosylhomocysteine
hydrolase
glutathione
Guanidinioacetate
methyltransferase
(GAMT)
N10-formyl-THF
formyl-GAR
(FGAR)
3 Pi
N5-methyl-THF
α-ketoglutarate
malate
+
TCA
H2O
Transaminase
B6
glutamate
SAH
Pi
THF
Homocysteine B12
methyltransferase
guanidinoacetate
α-aminoadipic semialdehyde
NAD+
Dehydrogenase
NAD+
Malate
dehydrogenase
succinyl-CoA
2 CO2
δ-ALA x2
porphobilinogen (PBG) x4
δ-ALA dehydratase
glutamate
heme b
β-hydroxybutyrate
2 H2O
NADP
saccharopine
NAD+
Saccharopine
NADH
dehydrogenase
glutamate
Transaminase
B6
cis-aconitate
NAD
protoporphyrinogen IX
B
Lactate dehydrogenase
glycinamide ribonucleotide
(GAR)
S-adenosylmethionine (SAM)
NAD+
Amidinotransferase
(in mitochondrial matrix)
Aspartate
transcarbamoylase
aspartate
Pi
NADP+
glycine
Lysine-αketoglutarate
reductase
+
α-ketoglutarate
Transaminase
SAM
carbamoyl phosphate
ADP
TCA
NADH
Glycine
cleavage
complex
THF
α-ketoglutarate
valine
α-ketoglutarate
α-ketoglutarate
CO2
NADH
oxaloacetate
-
Email questions or comments to: metabolicpathways@stanford.edu
ATP
+
dehydrogenase
δ-aminolevulinic acid (δ-ALA)
NH4+
NADPH
isoleucine
leucine
Alanine B6
transaminase (ALT)
pyruvate
NAD+
ADP
NADH
6
Pathways of
Human Metabolism
ADP
CoA
1
ATP
Pi
melatonin
Pi
2 ADP
5-phosphoribosylamine
glutamate
N5,N10-methylene-THF
Methenyl-THF
reductase
lysine
alanine
acetone
NADH
serine
glutamate
NADH
ATP
FADH2
β-hydroxybutyrate
+
N5,N10-methenyl-THF
Pi
H2O
Hydroxyindole
O-methyltransferase
Carbamoyl
phosphate
synthetase II
(CPSII)
glutamate
glutamate
GAR transformylase
methionine
NADPH
Serine hydroxymethyltransferase
(SHMT) B6
Branched chain amino acid breakdown
α-ketoglutarate
NADH
TCA
H2O
acetyl-CoA
pyrimidine synthesis
NADPH NADP+
Methenyl-THF
cyclohydrolase
sphingosine
cholesterol (cytosol)
(spontaneous)
3-phosphoserine
α-ketoglutarate
ATP
(n-2) fatty acyl-CoA acetyl-CoA
NAD
CoA
Enzyme cofactor:
methylmalonyl-CoA mutase and
homocysteine methyltransferase
acetoacetate
N10-formyl-THF
ceramide
(long chains only)
CO2
acetyl-CoA
B6
N-acetyl-5-HT
Methyltransferases
H2O
phosphatidylserine
CoA
(odd-chain fatty acids only)
HMG-CoA lyase (in liver)
TCA
Precursor: CoA
glutamate
Serotonin
N-acetyltransferase
2 ATP
ATP
fumarate
histidine catabolism
ADP Pi
C1-THF
synthase
Phosphoserine
phosphatase
Translocase
β-ketoacyl-CoA
β-hydroxy-β-methyl-glutaryl-CoA
(HMG-CoA)
Precursor:
NAD+/NADH and NADP+/NADPH
fatty acyl-carnitine
β-hydroxyacyl-CoA
acetoacetyl-CoA
acetyl-CoA
glutamate
(many cycles)
ATP
acetyl-CoA
3-phopsphopyruvate
NADH
NAD+
Pyruvate B7
carboxylase
B6
CPT1
fatty acyl-CoA
NADH
CoA
Phosphoserine
transaminase
acetyl-CoA
Fatty acyl-CoA synthetase
Thiolase
Phosphoglycerate
dehydrogenase
pyruvate
HCO3-
α-ketoglutarate
acetyl-CoA x2
acetate
acetoacetate
CO2
ATP
mitochondrial pyruvate
carrier
dicarboxylate
transporter
trans-enoyl-CoA
NAD+
DHFR
VMA
Fumarylacetaoacetate
hydrolase
H2O
ADP
CO2
PPi
acetaldehyde
Acetaldehyde
dehydrogenase
tetrahydrofolate
(THF)
α-ketoisocaproate
AST
Ketone body metabolism
Participates in additional reactions
dihydrofolate
Dihydrofolate
reductase
(DHFR)
NADH
Pyruvate
kinase
Malic enzyme
CoA
CPT2
acetaldehyde
folate
α-ketoglutarate
glutamate/
aspartate transporter
citrate
(spontaneous)
ATP AMP
B9
ATP
formate
vanillylmandelic acid (VMA)
Methionine adenosyltransferase
TCA
NADPH NADP+
PPi
SAH
5-hydroxyindoleacetic acid
THF
phosphoenolpyruvate
Phosphoenolpyruvate
carboxykinase
NADH
Malate
dehydrogenase
Aspartate
aminotransferase
B6 (AST)
CoA
BH2
Dihydrobiopterin
reductase
xylulose-5-phosphate
(step 1)
SAM
Monoamine
oxidase (MAO)
fumarylacetoacetate
α-ketoadipate
Long-chain fatty
acyl-CoA synthetase
CoA
(spontaneous)
CO2
B1
BH4
HCO3-
glutamine
GAR synthetase
CO2
NADPH
Pyrimidine synthesis
glutamine
Glutamine PRPP
amidotransferase (GPAT)
epinephrine
H2O
serotonin (5-HT)
niacin
Single-carbon metabolism and sulfur-containing amino acids
2-phosphoglycerate
2,3-bisphosphoglycerate
2,3-bisphosphoglycerate
phosphatase
Enolase
H2O
CO2
GTP GDP
glutamate
citrate
carnitine
NADH
GTP
O2
Aromatic amino acid
decarboxylase B6
(DOPA decarboxylase)
5-hydroxytryptophan
Maleylacetoacetate
isomerase
(spontaneous)
CO2
ATP
3-phosphoglycerate
Pi
dehydrogenase
3
glyceraldehyde3-phosphate
(step 2)
NAD+
Glyceraldehyde-3phosphate dehydrogenase
oxaloacetate
ADP
Citrate lyase
ATP
lysine
ethanol
NAD+
Alcohol
4
+
5
B1 +
Phosphoglycerate
mutase
Pi
acetyl-CoA
Pi
carnitine transporter
Nucleotides
6
erythrose4-phosphate
NADPH NADP+
Bisphosphoglycerate
mutase
CO2
-
Fatty acid synthase
ATP AMP
succinylacetoacetate
glyceraldehyde3-phosphate
Triosephosphate isomerase
Triose kinase ADP
ATP
Malonyl-CoA
decarboxylase
ATP
NADPH
CoA
H2O
norepinephrine
homovanillic acid (HVA)
H2O
Tryptophan
hydroxylase
NADP
Methyltransferase
glycine
tryptophan
Homogentisate
dioxygenase
Dopamine
β-hydroxylase
dopamine
BH2
Dihydrobiopterin
reductase
O2
maleylacetoacetate
1,3-bisphosphoglycerate
trans-enoyl-ACP
Enoyl-ACP
reductase
Ribulose-5phosphate
epimerase
BH4
+
GTP-cyclohydrolase
purine synthesis
CoA ACP
H2O
O2
formate
ribose-5-phosphate
Tyrosine
hydroxylase
NADP+ NADPH
homogentisate
(spontaneous)
M
5-phosphoribosyl pyrophosphate (PRPP)
Aromatic amino acid
decarboxylase B6
(DOPA decarboxylase)
Aldolase
malonyl-CoA
Pi
Acetyl-CoA ACP
transacylase
alkapton
Dihydroneopterin
triphosphate
ribulose-5-phosphate
B6
p-hydroxyphenylpyruvate
dioxygenase
CO2
CO2
Ribose-5phosphate
isomerase
7 5
+ + Transketolase
3 5
O2
succinylacetone
FADH2
NADP+
β-hydroxyacyl-ACP
CoA
B7
CO2
NADPH
β-ketoacyl-ACP
reductase
ATP
H2O
dihydroxyphenylanine
(L-DOPA)
p-hydroxyphenylpyruvate
(multiple steps)
(step 3)
glyceraldehyde
ACP
acetyl-ACP
AMP
5-phosphoribosyl pyrophosphate (PRPP)
Dihydrobiopterin (BH2)
Dihydrobiopterin
reductase
NADP+
NADPH
6-Phosphogluconate
dehydrogenase
O2
Transaminase
glutamate
L
Purine synthesis
α-ketoglutarate
Tetrahydrobiopterin (BH4)
6-phosphogluconate
(multiple steps)
tyrosine
α-ketoglutarate
K
melanin
Tyrosinase
(multiple steps)
H2O
Phenylalanine
hydroxylase
phenylalanine
H
6 7
+ + Transaldolase
4 3
fructose-1,6-bisphosphate
Fructose-1-P aldolase
(aldolase B)
fructose-1-phosphate
Malonyl-CoA ACP transacylase
β-ketoacyl-ACP
synthase
H2O
glutamate
B6
+
sedoheptulose7-phosphate
Transketolase
ADP
CO2
ACP
phenylpyruvate
O2
J
dopaquinone
triiodothyronine (T3)
thyroxine (T4)
NADP+ NADPH
PRPP synthetase
Pi
Fructose-1,6bisphophatase
Transaminase
NADP+ NADPH
Lactonase
Phosphomannose
isomerase
fructose-6-phosphate
mannose-6-phosphate
Hexokinase
NAD NADH
Fructokinase
(first cycle only)
ACP
Glutathione peroxidase
2 H2O
6-phosphogluconolactone
nucleotides
Phosphoglucose
isomerase
Dehydrogenase
phenyllactate
biosynthetic reduction reactions
Glucose-6-phosphate
dehydrogenase (G6PD)
Phosphoglycerate
kinase
malonyl-ACP
NADPH
NADP+
ATP
(multiple steps)
H2O2
Glutathione reductase
glutathione (oxidized)
ADP
Amino acids
Vitamins
B9
3'
sphingolipids
sphingosine
carnitine
Plasma membrane
biotin
4
+
ATP
CoA
free fatty acids
Inner mitochondrial membrane
B7
3
Glycerol-3-phosphate dehydrogenase
glycerol
3-phosphate
fructose
CO2
CoA
Outer mitochondrial membrane
B6
2'
Fatty acid synthesis
(7 cycles)
free fatty acids
Lipids
pyridoxal
phosphate
2
glucosamine- 6-phosphate
NADPH
β-ketoacyl-ACP
synthase
Pi
Oxidative
metabolism
pantothenate
Glycerol
kinase
palmitoyl-CoA
CoA
H2O
Carbohydrates
niacin
4
Phosphofructokinase-2
Fructose bisphosphatase-2
(Bifunctional enzyme)
FAD
fatty acyl-ACP
phosphatidic acid
Legend
riboflavin
3
ADP
estradiol
NAD NADH
(multiple steps)
CoA
Diet
B2
B3
B5
1
glutamate
CoA
formate
lipoproteins
(extrahepatic metabolism)
thiamine
2
(in mitochondrial matrix)
(n+2) fatty acyl-ACP
B1
0
Coenzyme Q (reduced)
NADP+
thin gray arrow
1
glutathione (reduced)
I
Aromatic amino acid metabolism
phenylacetate
ATP ADP
estrone
dihydrotestosterone (DHT)
NADP+
triacylglycerol
heavy gray arrow
Hexokinase or
glucokinase
fructose
Pentose phosphate pathway
Glucose-6-phosphatase
(in endoplasmic reticulum)
ATP
H
NADH
NAD+
fructose 2,6-bisphosphate
mannose
Galactokinase
(in peripheral tissue)
glycerol-3-phosphate
protein
modification
cell
lipoproteins
bile salts
(liver) membranes
gray dashed arrow
-1
ATP
galactose
Aldose reductase
+
11β-HSD1
serine
cholesterol
black dashed arrow
ADP
17β-HSD
Aromatase
4
α-1,6-Glucosidase
(in lysosomes)
Galactose-1-phosphate
uridyltransferase (GALT)
GAGs
Aromatase
3
+
+
7-Dehydrocholesterol
reductase
0
NADP+
Glycolysis and Gluconeogenesis
Lactose synthase
(inside Golgi of
mammary cells)
cortisone
7-dehydrocholesterol
vitamin D
(skin)
11β-hydroxysteroid
dehydrogenase 2 (11β-HSD2)
(in adrenal cortex, some in gonad)
8 NADPH
2 CO2
galactitol
5α reductase
aldosterone
10 O2
4 NADH
-2
glucose-1-phosphate
glycosaminoglycans (GAGs)
glycoproteins
NADP+ NADPH
glycolipids
androstenedione
17β-hydroxysteroid
dehydrogenase
(17β-HSD)
2
Mitochondrial glycerol
3-phosphate dehydrogenase (flavoprotein)
Aldosterone
synthase
lanosterol
4 NAD+
-1
galactose-1-phosphate
testosterone
cortisol
NADPH
-2
-3
UDP-galactose
glucose
+
dehydroepiandrosterone
(DHEA)
3β-hydroxysteroid
dehydrogenase
1
Phosphoglucomutase
11β-hydroxylase
corticosterone
squalene (C30)
NADP
11-deoxycortisol
11β-hydroxylase
NADP+
(multiple
steps)
17, 20 lyase
21-hydroxylase
(not in gonad)
11-deoxycorticosterone
(11-DOC)
NADPH
2 PPi
17-hydroxyprogesterone
21-hydroxylase
(not in gonad)
farnesyl pyrophosphate (FPP)
(C15) x2
Squalene synthase
3β-hydroxysteroid
dehydrogenase
17α-hydroxylase
progesterone
17, 20 lyase
17-hydroxypregnenolone
3β-hydroxysteroid
dehydrogenase
2 PPi
2 NAD
-3
-4
-5
UDP
lactose
Zona fasciculata and zona reticularis
3 ATP
(multiple
steps)
3
2 NADH
0
1 Glucose
1' (free)
UTP glucoseUTP 1-phosphate
uridyltransferase
UDP
UDP-glucose
dehydrogenase
cholesterol
2 NADP+
mevalonate
2
glycogenin
-4
-5
UDP-galactose epimerase
UDP-glucose
-1
1'
glycogen (n)
primed glycogenin
(8 cycles)
-2
4:4 Transferase
PPi
other
glucuronidation
reactions
β-hydroxy-β-methylglutaryl-CoA
(HMG-CoA)
-3
G
Sorbitol dehydrogenase
sorbitol
Pi
1'
-4
-5
UDP
conjugated
bilirubin
NADPH
3'
2'
UDP-glucose
7
Aldose reductase
4'
Debranching enzyme
Glycogen
phosphorylase
Glycogen
synthase
F
glucose
B6
(many cycles)
8
2
Pi
10
9
E
(branch point only) (debranching)
glycogen (n+1)
(branching)
7
10
Glycosyl (4:6)
transferase
(branching
1
enzyme)
D
(many cycles)
α-1,4 bonds
Thiolase
1
C
thymidine
deoxyuridine
dTTP
Thymidine kinase
deoxythymidine-5'-monophosphate (dTMP)
deoxyuridine 5’-monophosphate (dUMP)
ATP ADP
J
K
L
M
6
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