A
B
Cholesterol synthesis
Glycogen and galactose metabolism
acetyl-CoA x2
1
2
3
4
5
6
8
9
CoA
(many cycles)
9
acetoacetyl-CoA
acetyl-CoA
HMG-CoA synthase
CoA
2
3
4
5
α-1,6 bond
other
glucuronidation
reactions
Cholesterol
desmolase
17α-hydroxylase
pregnenolone
CO2
isopentenyl pyrophosphate (IPP)
(C5) x3
(multiple
steps)
Aldose reductase
Aromatase
Aromatase
11β-hydroxysteroid
dehydrogenase 1 (11β-HSD1)
glycerol
NAD NADH
ATP ADP
fructose
serine
glycerol-3-phosphate
protein
modification
8 NADP
+
(multiple
steps)
Glycerol-3-phosphate
acyltransferase
(GPAT)
(multiple steps)
protein
modification
fatty acyl-ACP
β-ketoacyl-ACP
synthase
formate
CoA
NADPH
phosphatidic acid
NADP+
AMP
H2O
Pi
Triacylglycerol
synthetase
complex
ATP
phospholipids
1,2-diacylglycerol
Long-chain
fatty
acyl-CoA
synthetase
acetyl-ACP
β-ketoacyl-ACP
β-hydroxyacyl-ACP
dehydratase
palmitate (C16)
CoA
lipoproteins
(extrahepatic metabolism)
Legend
Nucleotides
Heme
metabolism
free fatty acids
4
B9
B12
biotin
folate
cobalamin
tricarboxylate
transporter
PPi
NAD
+
NADP+ NADPH
malate
fatty acyl- CoA
Long-chain fatty
acyl-CoA synthetase
ATP AMP
fatty acids ≤ C12
acetaldehyde
aspartate
Acyl-CoA
Synthetase
Acetyl-CoA
synthetase
5
PPi
Acyl-CoA
dehydrogenases
Hydroxyacyl-CoA
dehydrogenases
AMP
HMG-CoA synthase (in liver)
CoA
Thiolase
β-hydroxy-β-methyl-glutaryl-CoA
(HMG-CoA)
Precursor:
NAD+/NADH and NADP+/NADPH
acetyl-CoA
Enzyme cofactor:
transaminases and other reactions
involving amino acids
Heme synthesis
Enzyme cofactor: carboxylases
glycine
Single-carbon carrier
succinyl-CoA
CO2
glutamate
FAD
NADH
NAD+
δ-aminolevulinic
acid synthase B6
VERSION 10.16
NADH
H2O
Copyright 2016 Stanford School of Medicine. All rights reserved.
This work is licensed under a Creative Commons AttributionNonCommercial-NoDerivatives 4.0 International License.
NAD
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)
Propionyl-CoA carboxylase
B7
Citrate
synthase
oxaloacetate
CoA
H2O
Malate
dehydrogenase
Aconitase
ADP
CoA
NH4
Fumarase
HCO3
CoA
α-ketoglutarate
B
dehydrogenase 1
fumarate
Thiolase
acetyl-CoA x2
3 O2
Protoporphyrinogen
IX oxidase
Succinyl-CoA
synthetase GDP
many
amino Transaminases
acids
NADH
4 CO2
hydroxymethylbilane
uroporphyrinogen III
Uroporphyrinogen III
Uroporphyrinogen
4 NH4+
synthase
decarboxylase
D
Succinic semialdehyde
dehydrogenase
CoA
Glutamate
decarboxylase
2 H2O
Coproporphyrinogen oxidase
-
+
+
4H
+
I
-
+
-
+
-
+
-
-
+
-
+
FADH2
II
- - - - -
-
+
+
2 e-
FAD
- - -
-
-
-
-
-
-
+
+
+
+
+
+
+
+
+
-
-
2e
CoQ
NADH
dehydrogenase
(Complex I)
-
+
+
-
-
-III -
-
-
-
-- -
2e
-
-
-
-
-
2e
-
-
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
Pi
-
IV
+
+
-
+
Cytochrome c
oxidase
(Complex IV) 2 H+
+
-
+
-
+
-
-
+
-
+
ATP synthase
(Complex V)
+
3 H+
+
-
+
-
-
phosphate
transporter
+
+
Pi
-
-
-
-
-
H2O
adenine nucleotide
transporter
+
UDP-glucuronate
NADPH NADP+
biliverdin
+
+
ADP
+
NH4+
+
Pi
Biliverdin reductase
3 O2 Fe 3+ CO
Heme oxygenase
F
AMP
deaminase
ATP
conjugated bilirubin
UDP-glucuronyltransferase
Nucleotidase
(deoxy)
ribose-1-P
(deoxy)
adenosine
Adenosine
deaminase
H2O
Guanine
deaminase
G
H2O
NH4
sulfite (SO3 )
bile salts
urine
Arginase
urea
IMP cyclohydrolase
ATP
arginine
NH4+
H2O
H2O
UMP
Nucleotidase
(deoxy)
inosine
adenylosuccinate
PPi
PRPP
Proline oxidase
AMP
(deoxy)
ribose-1-P
Thymidine
phosphorylase
NADPH
NADP+
Pi
dADP
Pi
H2O
Phosphatase
Pi
CDP
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
deoxy
ribose-1-P
CO2
dCDP
dGDP
ATP
Kinase
ADP
ATP
Kinase
ADP
dATP
thymine
Dihydropyrimidine
dehydrogenase
(DPD)
β-ureidopropionate
ribose-1-P
uracil
Pi
Uridine phosphorylase
GDP
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 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
GDP
Dihydropryimidinase
H2O2
UTP
(deoxy)
ribose-1-P
dihydrouracil
H2O
CTP
H2O
Phosphatase
Pi
ADP
thymidine
uracil
H2O2
Adenylate
kinase
Hypoxanthine-guanine
phosphoribosyltransferase
(HGPRT)
hypoxanthine
Adenine
phosphoribosyltransferase
proline
NADP+
GMP reductase
NADPH
XMP
NADP+
GTP
H2O
Phosphatase
Pi
deoxyTMP
H2O
H2O
Nucleotidase
Pi
Pi
(deoxy)
uridine
Pi
O2
NAD+
IMP dehydrogenase
NADH
4
+
Uridine
phosphorylase
H2O
H2O
fumarate
pyrroline-5-carboxylate
NH4+
Base salvage
H2O
pyrroline5-carboxylate
H2O
Adenylosuccinate
GTP
synthetase
GDP
Pi
Adenylosuccinate
lyase
NADPH
CTP
inosine monophosphate
(IMP)
AMP
asparagine
fumarate
(spontaneous)
glutamate
aspartate
PPi
NH4+
glutamine
formaminoimidazole carboxamide ribonucleotide
(FAICAR)
α-ketoglutarate
NH4+
Asparaginase
H2O
Argininosuccinate
lyase
(spontaneous)
(deoxy)
cytidine Pyrimidine
nucleoside
deaminase
uric acid
H
ATP
CTP synthetase
ADP + Pi
N10-formyl-THF
AICAR transformylase
aspartate
3
UTP
aminoiminidazole carboxamide ribonucleotide
(AICAR)
sulfate (SO42-)
NH4+
AMP deaminase
Nucleoside
diphosphate
kinase
Adenylosuccinate lyase
fumarate
Sulfite
oxidase
taurine
ATP
NH4
+
Pi
Xanthine
oxidase
Xanthine oxidase
bile
UDP
H2O
2-
glutamine
Asparagine synthetase
glutamate
NADPH
O2
ornithine
Pi
(deoxy) IMP
Pi
xanthine
feces
bilirubin
B6
ATP
Nucleotidase
ATP
ADP
Pi
-
glutamate
glutamate
Transaminase
Argininosuccinate
synthetase
PPi
ATP
ornithine/citrulline
transporter
(deoxy) CMP
H2O
Nucleotidase
guanine
ADP
SAICAR synthetase
ADP
pyruvate
β-sulfinylpyruvate
Deoxyribonucleotides
(deoxy) AMP
O2
3 NADPH 3 NADP+
ornithine
glutamic semialdehyde
Purine nucleoside
phosphorylase
-
B6
oxaloacetate
argininosuccinate
RNA/DNA
(deoxy) guanosine
Purine
nucleoside
phosphorylase
UMP kinase
aspartate
ADP
NADH
NADP+
H2O
Pi
hypoxanthine
-
hypotaurine
ATP
Pi
Transaminase
Hypotaurine
dehydrogenase
Nitric oxide
synthase
Pi
Ornithine
transcarbamoylase (OTC)
NADPH NADP+
Pyrroline-5carboxylate synthetase
Pi
urea cycle (regulation)
V
CO2
AMP
citrulline
Pi
glutamate
H2O
+
-
H2O
uridine-5'-monophosphate
(UMP)
succinylaminoimidazole carboxamide ribonucleotide
(SAICAR)
α-ketoglutarate
NAD+
ornithine/citrulline
transporter
ATP ADP
ATP
ADP + Pi
2 H+
-
creatinine
(excreted)
citrulline
Ornithine transaminase B6
aspartate
Dehydrogenase
Nucleotidase
3 H+
B6
Cytochrome c
reductase
(Complex III) 4 H+
heme b
E
-
ADP
acetate H2O
Hydrolase
γ-aminobutyric
GABA transaminase acid (GABA)
Cyt c
Succinate
dehydrogenase
(Complex II)
Heme degradation
coproporphyrinogen III
-
CO2
α-ketoglutarate
/2 O2 + 2H
I
(spontaneous)
H2O
polyamines
Nucleotide breakdown
N-acetylglutamate
Electron transport chain
4 H+
succinate
Cysteine sulfinite
decarboxylase
TCA
PPi
N-acetylglutamate synthase
acetyl-CoA CoA
many
α-ketoacids
1
protoporphyrinogen IX
O2
succinic semialdehyde
NADH
NAD
Creatine
kinase
oxaloacetate
(deoxy) GMP
NH4+ H2O
ATP
Glutamine synthetase
B6
glutamate
NAD+
GTP
+
phosphocreatine
NO
Hydratase
glutamine
NADH NAD
Glutamate
dehydrogenase-1
NADH
succinyl-CoA
Pi
4 H+
ADP
ATP
B6
glutamate
NAD+
CO2
3 H2O2
Pi
Nucleases
H2O
NH4
cysteine
sulfinate
SAM
succinyl-CoA
OMP decarboxylase
(UMP synthase)
CO2
CO2
ATP
Thiolase
acetyl-CoA x2
Glutaminase
NH4+ H2O
nucleotide salvage
Threonine dehydratase
+
threonine
acetoacetyl-CoA
TCA
O2
Cysteine
dioxygenase
2 ADP
carbamoyl
phosphate
NADH
CoA
+
2 H+
CoA
Methylmalonyl-CoA
mutase B12
succinyl-CoA
α-ketoglutarate
-
propionyl-CoA
ornithine
crotonyl-CoA
NAD+
aminoimidazole ribonucleotide
(AIR)
H2O
cysteine
Coenzyme A
2 ATP
β-hydroxybutyryl CoA
methylmalonyl-CoA
Pi
(in mitochondrial matrix)
α-ketobutyrate
HCO3
Carbamoyl
phosphate
synthetase I
(CPS1)
Glutaryl-CoA
dehydrogenase
H2O
Propionyl-CoA
carboxylase B7
ADP
Ammonium capture and release
+
odd-chain fatty
acyl-CoA
(last three carbons only)
HCO3-
ATP
acetyl-CoA
acetoacetate
NADH
CO2
H2O
glutathione
orotidine-5'-monophosphate (OMP)
carboxyaminoimidazole ribonucleotide
(CAIR)
Cystathionine
γ-lyase B6
porphyrins
ATP
ADP
-
glutaryl-CoA
FAD
HMG-CoA lyase
NAD
NADH
isobutyryl-CoA
propionyl-CoA
º-hydroxy-β-methylglutaryl-CoA
(HMG-CoA)
+
B1
AIR synthetase
AIR carboxylase
Urea cycle
NH4
2
Orotate
phosphoribosyltransferase
PPi
THF
+
Dihydroorotate
dehydrogenase
(in mitochondria)
NADH
formiminoglycinamidine ribonucleotide
(FGAM)
cystathionine
B6
CoA
α-ketoadipate B1
dehydrogenase
CO2
FADH2
β-methylglutaconyl-CoA
hydratase
H2O
NADH
NAD+
acetyl-CoA
β-methylglutaconyl-CoA
cis-aconitate
B1
CoA
BCKAD
CO2
CH3 (used for methylation)
adenosine
serine
Cystathionine
β-synthase B6
creatine
α-ketoadipate
(multiple steps)
β-methylcrotonyl-CoA
carboxylase B7
Aconitase
NAD+
ADP
PRPP
S-adenosylhomocysteine (SAH)
(spontaneous)
Transaminase
NAD+
orotate
S-adenosylhomocysteine
hydrolase
nucleotides
SAH
dihydroorotate
H2O
glutamine
homocysteine
NADH
α-aminoadipate
glutamate
α-ketoisovalerate
α-methylbutyryl-CoA
HCO3-
ADP
H2O
Isocitrate
dehydrogenase
ATP
2 CO2
H2O
NADH
β-methylcrotonyl-CoA
citrate
CoA
BCKAD
CO2
NAD+
Isovaleryl-CoA
dehydrogenase
NADH
Ferrochelatase
C
α-ketoisovalerate
α-keto-β-methylglutarate
isovaleryl-CoA
Fe2+
protoporphyrin IX
PBG deaminase
B6
glutamate
α-keto-β-methylglutarate
α-ketoisocaproate
NAD+
B12
NADH
3 Pi
N5-methyl-THF
α-ketoglutarate
Citric acid cycle (tricarboxylic acid cycle)
methylmalonyl-CoA
TCA
H2O
B6
glutamate
heme b
β-hydroxybutyrate
δ-ALA x2
porphobilinogen (PBG) x4
δ-ALA dehydratase
glutamate
Transaminase
Methylene-THF
reductase
arginine
Guanidinioacetate
methyltransferase
(GAMT)
Dihydroorotase
formyl-GAR
(FGAR)
FGAM synthetase
Homocysteine B12
methyltransferase
guanidinoacetate
α-aminoadipic semialdehyde
NAD+
Dehydrogenase
valine
NAD+
malate
+
dehydrogenase
2 H2O
NADP
saccharopine
NAD+
Saccharopine
NADH
dehydrogenase
glutamate
+
Transaminase
B6
isocitrate
protoporphyrinogen IX
B
lactate
Lysine-αketoglutarate
reductase
NADPH
α-ketoglutarate
Transaminase
Lactate dehydrogenase
+
succinyl-CoA
Methylmalonyl-CoA mutase
THF
S-adenosylmethionine (SAM)
glutathione
Amidinotransferase
(in mitochondrial matrix)
N-carbamoyl aspartate
Pi
NADP+
glycine
α-ketoglutarate
Pi
N10-formyl-THF
TCA
NAD+
THF
Aspartate
transcarbamoylase
aspartate
glycinamide ribonucleotide
(GAR)
5-hydroxyindoleacetic acid
NADH
Glycine
cleavage
complex
lysine
α-ketoglutarate
α-ketoglutarate
CO2
NADH
oxaloacetate
-
Email questions or comments to: [email protected]
ATP
pyruvate
NAD+
ADP
NAD
β-hydroxybutyrate
δ-aminolevulinic acid (δ-ALA)
serine
isoleucine
leucine
Alanine B6
aminotransferase (ALT)
NADH
ATP
FADH2
6
Pathways of
Human Metabolism
ADP
acetone
NADH
NH4+
carbamoyl phosphate
ADP
Pi
SAH
1
ATP
glutamate
N5,N10-methylene-THF
Methenyl-THF
reductase
Pi
2 ADP
5-phosphoribosylamine
melatonin
Carbamoyl
phosphate
synthetase II
(CPSII)
glutamate
glutamate
GAR transformylase B9
methionine
NADPH
Serine hydroxymethyltransferase
(SHMT) B6
alanine
glutamate
Hydroxyindole
O-methyltransferase
SAM
pyrimidine synthesis
N5,N10-methenyl-THF
Pi
H2O
VMA
2 ATP
ATP
fumarate
NADPH NADP+
Methenyl-THF
cyclohydrolase
Branched chain amino acid breakdown
α-ketoglutarate
cholesterol (cytosol)
+
3-phosphoserine
NADH
TCA
(n-2) fatty acyl-CoA acetyl-CoA
(spontaneous)
N10-formyl-THF
sphingosine
ATP
(odd-chain fatty acids only)
NAD
CoA
Enzyme cofactor:
methylmalonyl-CoA mutase and
homocysteine methyltransferase
acetoacetate
B6
N-acetyl-5-HT
Methyltransferases
H2O
ceramide
(long chains only)
CO2
Precursor: CoA
ADP Pi
C1-THF
synthase
Phosphoserine
phosphatase
α-ketoglutarate
CoA
β-ketoacyl-CoA
HMG-CoA lyase (in liver)
DHFR
Translocase
β-hydroxyacyl-CoA
acetoacetyl-CoA
TCA
acetoacetate
histidine catabolism
phosphatidylserine
(many cycles)
ATP
acetyl-CoA
Enzyme cofactor:
α-ketoacid dehydrogenases and
transketolase
Precursor:
FAD/FADH2
fatty acyl-carnitine
fatty acyl-CoA
Hydratase
acetyl-CoA
glutamate
acetyl-CoA
NADH
CoA
Phosphoserine
transaminase
3-phosphopyruvate
NADH
NAD+
Pyruvate B7
carboxylase
acetyl-CoA x2
CoA
Phosphoglycerate
dehydrogenase
pyruvate
PPi
Fatty acyl-CoA synthetase
Thiolase
Dihydrofolate
reductase
(DHFR)
tetrahydrofolate
(THF)
vanillylmandelic acid (VMA)
Fumarylacetaoacetate
hydrolase
H2O
CO2
ATP
CO2
HCO3-
α-ketoglutarate
B6
CPT1
BH2
Dihydrobiopterin
reductase
fumarylacetoacetate
ADP
mitochondrial pyruvate
carrier
dicarboxylate
transporter
trans-enoyl-CoA
NAD+
dihydrofolate
ATP
formate
α-ketoisocaproate
AST
CPT2
CoA
folate
NADH
Pyruvate
kinase
Malic enzyme
CoA
(spontaneous)
NADH
B9
α-ketoglutarate
glutamate/
aspartate transporter
citrate
carnitine
acetate
Crosses membrane
Vitamins
B7
dehydrogenase
Acetaldehyde
dehydrogenase
Multiple cycles
pantothenate
Aspartate
aminotransferase
B6 (AST)
aspartate
BH4
HCO3-
glutamine
PPi
SAH
Methionine adenosyltransferase
TCA
NADPH NADP+
epinephrine
Monoamine
oxidase (MAO)
xylulose-5-phosphate
(step 1)
SAM
Aromatic amino acid
Serotonin
B
decarboxylase 6
N-acetyltransferase
5-hydroxyserotonin (5-HT)
tryptophan
acetyl-CoA CoA
CO2
NADPH
THF
phosphoenolpyruvate
Phosphoenolpyruvate
carboxykinase
NADH
Malate
dehydrogenase
glutamate
CoA
H2O
Single-carbon metabolism and sulfur-containing amino acids
3-phosphoglycerate
oxaloacetate
Fatty acid oxidation
lysine
ethanol
NAD+
Alcohol
(spontaneous)
CO2
norepinephrine
H2O
homovanillic acid (HVA)
niacin
NADP
Pyrimidine synthesis
glutamine
Glutamine PRPP
amidotransferase (GPAT)
glycine
Tryptophan
hydroxylase
tryptophan
Maleylacetoacetate
isomerase
(spontaneous)
α-ketoadipate
acetaldehyde
Participates in additional reactions
niacin
acetyl-CoA
citrate
Ketone body metabolism
Inner mitochondrial membrane
B6
fatty acids
> C24 to peroxisomes
fatty acids
> C14 and < C22
GTP
B1
O2
maleylacetoacetate
succinylacetoacetate
O2
Methyltransferase
GAR synthetase
Homogentisate
dioxygenase
H2O
CO2
BH2
Dihydrobiopterin
reductase
Dopamine
β-hydroxylase
dopamine
ATP
2-phosphoglycerate
2,3-bisphosphoglycerate
2,3-bisphosphoglycerate
phosphatase
Enolase
H2O
CO2
GTP GDP
CO2
ADP
Citrate lyase
ATP
ATP AMP
CoA
3
glyceraldehyde3-phosphate
(step 2)
NAD+
Glyceraldehyde-3phosphate dehydrogenase
Pi
Outer mitochondrial membrane
pyridoxal
phosphate
fatty acids
≤ C12
Multiple steps
riboflavin
HCO3
-
Pi
carnitine transporter
Lipids
B2
B3
B5
Pi
Amino acids
Oxidative
metabolism
thiamine
Malonyl-CoA
decarboxylase
ATP
NADPH
Enoyl-ACP
reductase
carnitine
Carbohydrates
Acetyl-CoA
carboxylase
trans-enoyl-ACP
free fatty acids
Diet
ADP
Fatty acid synthase
H2O
4
+
5
Phosphoglycerate
mutase
Pi
NADP+
β-hydroxyacyl-ACP
erythrose4-phosphate
NADPH NADP+
Bisphosphoglycerate
mutase
CoA ACP
CO2
NADPH
β-ketoacyl-ACP
reductase
GTP-cyclohydrolase
Ribulose-5phosphate
epimerase
BH4
+
1,3-bisphosphoglycerate
malonyl-CoA
Pi
Acetyl-CoA ACP
transacylase
O2
DOPA B6
decarboxylase
NADP+ NADPH
homogentisate
(spontaneous)
formate
glyceraldehyde3-phosphate
Triosephosphate isomerase
Triose kinase ADP
ATP
ADP
CO2
ACP
CoA
fatty acyl-CoA
(first cycle only)
ACP
(7 cycles)
PPi
B7
(n+2) fatty acyl-ACP
B1
dihydroxyacetone
phosphate (DHAP)
glyceraldehyde
ACP
CoA
palmitoyl-CoA
CoA
Ribose-5phosphate
isomerase
7 5
+ + Transketolase
3 5
alkapton
Dihydroneopterin
triphosphate
ribulose-5-phosphate
B6
p-hydroxyphenylpyruvate
dioxygenase
CO2
CO2
purine synthesis
Fructose-1-P aldolase
(aldolase B)
fructose-1-phosphate
Malonyl-CoA ACP transacylase
malonyl-ACP
(multiple steps)
ribose-5-phosphate
Tyrosine
hydroxylase
Aldolase
Phosphoglycerate
kinase
CO2
6
B1 +
fructose-1,6-bisphosphate
sphingolipids
sphingosine
ATP
O2
M
5-phosphoribosyl pyrophosphate (PRPP)
H2O
dihydroxyphenylanine
(L-DOPA)
p-hydroxyphenylpyruvate
succinylacetone
Transketolase
Fatty acid synthesis
CoA
ADP
5-phosphoribosyl pyrophosphate (PRPP)
Dihydrobiopterin (BH2)
Dihydrobiopterin
reductase
NADP+
NADPH
(step 3)
NAD NADH
ATP
NADP+
thin gray arrow
ADP
Coenzyme Q (oxidized)
+
Fructokinase
(in peripheral tissue)
triacylglycerol
heavy gray arrow
Glutamine:F6P
transaminase B6
Glycerol-3-phosphate dehydrogenase
glycerol
3-phosphate
NADPH
NADP+
Lactonase
O2
Transaminase
glutamate
L
Purine synthesis
α-ketoglutarate
Tetrahydrobiopterin (BH4)
6-phosphogluconate
6 7
+ + Transaldolase
4 3
FADH2
FAD
estradiol
dihydrotestosterone (DHT)
H
(multiple steps)
tyrosine
α-ketoglutarate
K
melanin
Tyrosinase
(multiple steps)
H2O
Phenylalanine
hydroxylase
phenylalanine
+
PRPP synthetase
Pi
Fructose-1,6bisphophatase
ATP
Phosphofructokinase-1
(in mitochondrial matrix)
+
11β-HSD2
cell
lipoproteins
bile salts
(liver) membranes
gray dashed arrow
glutamine
glucosamine- 6-phosphate
Coenzyme Q (reduced)
Glycerol
kinase
glutamate
B6
J
dopaquinone
triiodothyronine (T3)
thyroxine (T4)
NADP+ NADPH
sedoheptulose7-phosphate
+
cholesterol
black dashed arrow
glutamate
17β-HSD
phenylpyruvate
6-Phosphogluconate
dehydrogenase
nucleotides
Transaminase
NADP+ NADPH
H2O
6-phosphogluconolactone
O2
ATP ADP
estrone
cortisone
7-dehydrocholesterol
vitamin D
(skin)
ATP
galactose
Dehydrogenase
Glutathione peroxidase
2 H2O
Phosphomannose
isomerase
fructose-6-phosphate
mannose-6-phosphate
Hexokinase
mannose
Galactokinase
GAGs
androstenedione
17β-hydroxysteroid
dehydrogenase
(17β-HSD)
(in adrenal cortex, some in gonad)
8 NADPH
7-Dehydrocholesterol
reductase
galactitol
5α reductase
aldosterone
10 O2
2 CO2
dehydroepiandrosterone
(DHEA)
3β-hydroxysteroid
dehydrogenase
ADP
(multiple steps)
phenyllactate
biosynthetic reduction reactions
Glucose-6-phosphate
dehydrogenase (G6PD)
Phosphoglucose
isomerase
ATP
ADP
Mitochondrial glycerol
3-phosphate dehydrogenase (flavoprotein)
Aldosterone
synthase
lanosterol
4 NADH
glycosaminoglycans (GAGs)
glycoproteins
NADP+ NADPH
glycolipids
NADPH
I
Aromatic amino acid metabolism
phenylacetate
H2O2
Glutathione reductase
glutathione (oxidized)
ADP
glucose-6-phosphate
Phosphofructokinase-2
Fructose bisphosphatase-2
(Bifunctional enzyme)
galactose-1-phosphate
testosterone
cortisol
NADPH
4 NAD+
UDP
lactose
+
glutathione (reduced)
fructose 2,6-bisphosphate
Galactose-1-phosphate
uridyltransferase (GALT)
Lactose synthase
(inside Golgi)
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
glucose
fructose
Pentose phosphate pathway
Glycolysis and Gluconeogenesis
UDP-galactose
UDP
Zona fasciculata and zona reticularis
3 ADP
3
2 NADH
3 ATP
(multiple
steps)
UDP-glucose
UDP-glucose
dehydrogenase
UDP-glucuronate
mevalonate
2
glycogenin
Phosphoglucomutase
H
NADH
NAD+
NADP+
UDP-galactose epimerase
(8 cycles)
cholesterol
2 NADP+
UTP
UTP glucose1-phosphate
uridyltransferase
glucose-1-phosphate
PPi
primed glycogenin
Zona glomerulosa
2 NADPH
CoA
H2O
Hexokinase or
glucokinase
glycogen (n)
ADP
NADP+
Glucose-6-phosphatase
(in endoplasmic reticulum)
ATP
G
Sorbitol dehydrogenase
sorbitol
Pi
Glycogen
phosphorylase
2 Pi
conjugated
bilirubin
β-hydroxy-β-methylglutaryl-CoA
(HMG-CoA)
NADPH
B6
7
6
Aldose reductase
glucose
ATP
glycogen (n)
8
Steroid hormone synthesis
HMG-CoA reductase
Glycogen
synthase
F
Pi
UDP
10
E
(branch point only)
4:4 transferase and α-1,6-glucosidase (debranching enzyme)
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