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Acetyl-CoA
acetyltransferase,
cytosolic
Mevalonate
kinase
Phosphomevalonate
kinase
Diphosphomevalonate
decarboxylase
Isopentenyl-
diphosphate
Delta-isomerase
1
Farnesyl
pyrophosphate
synthase
Geranylgeranyl
pyrophosphate
synthase
Farnesyl
pyrophosphate
synthase
Geranylgeranyl
pyrophosphate
synthase
Squalene
synthase
Squalene
monooxygenase
Lanosterol
synthase
Delta(24)-
sterol
reductase
Lanosterol
14-alpha
demethylase
Lamin-B
receptor
Delta(24)-
sterol
reductase
Methylsterol
monooxygenase 1
Sterol-4-
alpha-
carboxylate 3-
dehydrogenase,
decarboxylating
3-keto-steroid
reductase
3-beta-
hydroxysteroid-
Delta(8),Delta(7)-
isomerase
Delta(24)-
sterol
reductase
Delta(24)-
sterol
reductase
3-beta-
hydroxysteroid-
Delta(8),Delta(7)-
isomerase
3-beta-
hydroxysteroid-
Delta(8),Delta(7)-
isomerase
Lathosterol
oxidase
Delta(24)-
sterol
reductase
Delta(24)-
sterol
reductase
3-hydroxy-3-
methylglutaryl-
coenzyme A
reductase
Sterol O-
acyltransferase
1
Lysosomal acid
lipase/cholesteryl
ester hydrolase
Acetoacetyl-CoA
Acetyl-CoA
Dissipation
Cholesterol
CoA
H2 O
3-Hydroxy-3-methylglutaryl-CoA
CoA
Mevalonic acid
ATP
Mevalonic acid-5P
ADP
ATP
ADP
(S)-5-Diphosphomevalonic acid
ATP
ADP
Pi
Isopentenyl pyrophosphate
CO2
Dimethylallylpyrophosphate
Geranyl-PP
PPi
Farnesyl pyrophosphate
PPi
NADPH
Squalene
NADP
PPi
O2
NADPH
(S)-2,3-Epoxysqualene
H2 O
NADP
Lanosterol
NADPH
24,25-Dihydrolanosterol
NADP
O2
NADPH
4,4-Dimethylcholesta-
8,14,24-trienol
Formic acid
NADP
H2 O
NADPH
4,4-Dimethyl-5a-cholesta-
8,24-dien-3-b-ol
NADP
NADPH
4,4-Dimethyl-5a-cholesta-8-
en-3b-ol
NADP
NADPH
O2
4a-Methylzymosterol-4-
carboxylic acid
NADP
H2 O
NADP
3-Keto-4-methylzymosterol
NADPH
CO2
NADPH
4a-Methylzymosterol
NADP
Zymosterol intermediate 2
5a-Cholesta-7,24-dien-3b-ol
NADPH
5a-Cholest-8-en-3b-ol
NADP
NADPH
Lathosterol
NADP
7-Dehydrodesmosterol
Accumulation
7-Dehydrocholesterol
NADPH
NADP
Desmosterol
NADPH
O2
NADP
H2 O
NADPH
NADP
NADPH
NADP
Vitamin D3
Accumulation
8-Dehydrocholesterol
NADPH
NADP
CoA
Fatty acid
Cholesterol Ester
H2 O
Fatty acid
H2 O
Magnesium
Magnesium
Magnesium
Magnesium
Magnesium
Magnesium
FAD
FAD
Heme
FAD
Iron
FAD
FAD
Iron
FAD
FAD
Butyrate
Metabolism
Pyruvate
Metabolism
Valine,
Leucine, and
Isoleucine
Degradation
Tryptophan
Metabolism
Pyruvate
Metabolism
Fatty Acid
Metabolism
Steroidogenesis
Bile Acid
Biosynthesis
Endoplasmic Reticulum
Peroxisome
Peroxisome
Intracellular Space
Extracellular Space
Endoplasmic Reticulum
Lysosome
hv
Spontaneous isomeration
Acat2
Mvk
Pmvk
Mvd
Idi1
Fdps
Ggps1
Fdps
Ggps1
Fdft1
Sqle
Lss
Dhcr24
Cyp51a1
Lbr
Dhcr24
Msmo1
Nsdhl
Hsd17b7
Ebp
Dhcr24
Dhcr24
Ebp
Ebp
Sc5d
Dhcr24
Dhcr24
Hmgcr
Soat1
Lipa
Acetoacetyl-CoA
Acetyl-CoA
Dissipation
Cholesterol
Coenzyme A
Water
3-Hydroxy-3-
methylglutaryl-
CoA
Coenzyme A
Mevalonic acid
Adenosine
triphosphate
Mevalonic
acid-5P
Adenosine
diphosphate
Adenosine
triphosphate
Adenosine
diphosphate
(S)-5-
Diphosphomevalonic
acid
Adenosine
triphosphate
Adenosine
diphosphate
Phosphate
Isopentenyl
pyrophosphate
Carbon dioxide
Dimethylallylpyrophosphate
Geranyl-PP
Pyrophosphate
Farnesyl
pyrophosphate
Pyrophosphate
NADPH
Squalene
NADP
Pyrophosphate
Oxygen
NADPH
(S)-2,3-
Epoxysqualene
Water
NADP
Lanosterol
NADPH
24,25-
Dihydrolanosterol
NADP
Oxygen
NADPH
4,4-
Dimethylcholesta-
8,14,24-
trienol
Formic acid
NADP
Water
NADPH
4,4-Dimethyl-
5a-cholesta-
8,24-dien-3-
b-ol
NADP
NADPH
4,4-Dimethyl-
5a-cholesta-
8-en-3b-ol
NADP
NADPH
Oxygen
4a-
Methylzymosterol-
4-carboxylic
acid
NADP
Water
NADP
3-Keto-4-
methylzymosterol
NADPH
Carbon dioxide
NADPH
4a-
Methylzymosterol
NADP
Zymosterol
intermediate 2
5a-Cholesta-
7,24-dien-3b-
ol
NADPH
5a-Cholest-8-
en-3b-ol
NADP
NADPH
Lathosterol
NADP
7-
Dehydrodesmosterol
Accumulation
7-
Dehydrocholesterol
NADPH
NADP
Desmosterol
NADPH
Oxygen
NADP
Water
NADPH
NADP
NADPH
NADP
Vitamin D3
Accumulation
8-
Dehydrocholesterol
NADPH
NADP
Coenzyme A
Fatty acid
Cholesterol
Ester
Water
Fatty acid
Water
Butyrate
Metabolism
Pyruvate
Metabolism
Valine,
Leucine, and
Isoleucine
Degradation
Tryptophan
Metabolism
Pyruvate
Metabolism
Fatty Acid
Metabolism
Steroidogenesis
Bile Acid
Biosynthesis
Acat2
Mvk
Pmvk
Mvd
Idi1
Fdps
Ggps1
Fdps
Ggps1
Fdft1
Sqle
Lss
Dhcr24
Cyp51a1
Lbr
Dhcr24
Msmo1
Nsdhl
Hsd17b7
Ebp
Dhcr24
Dhcr24
Ebp
Ebp
Sc5d
Dhcr24
Dhcr24
Hmgcr
Soat1
Lipa
ActaCoA
Ac-CoA
Dissipation
Lanol
CoA
H2 O
HMG-CoA
CoA
Mva
ATP
Mvn-5P
ADP
ATP
ADP
S-5DPMA
ATP
ADP
Pi
IsopPyr
CO2
Ipe
Gryl-PP
PPi
FarPP
PPi
NADPH
Squalen
NADP
PPi
O2
NADPH
S23O
H2 O
NADP
Lastrol
NADPH
2425Di
NADP
O2
NADPH
FF-Mas
Formate
NADP
H2 O
NADPH
44Dcdol
NADP
NADPH
44DCBol
NADP
NADPH
O2
4M4CA
NADP
H2 O
NADP
3K4Mzol
NADPH
CO2
NADPH
4aMzmol
NADP
ZymolI2
5CDBol
NADPH
5aChlol
NADP
NADPH
Lathost
NADP
7-DHMOL
Accumulation
7DHC
NADPH
NADP
Desmost
NADPH
O2
NADP
H2 O
NADPH
NADP
NADPH
NADP
VitD3
Accumulation
8Dhchol
NADPH
NADP
CoA
FA
ChoEst
H2 O
FA
H2 O
Mg2+
Mg2+
Mg2+
Mg2+
Mg2+
Mg2+
FAD
FAD
Heme
FAD
Iron
FAD
FAD
Iron
FAD
FAD
PW000014
PW000054
PW000051
PW000163
PW000054
PW000023
PW000141
PW000145
Endoplasmic Reticulum
Peroxisome
Peroxisome
Intracellular Space
Extracellular Space
Endoplasmic Reticulum
Lysosome
hv
Spontaneous isomeration
Acat2
Mvk
Pmvk
Mvd
Idi1
Fdps
Ggps1
Fdps
Ggps1
Fdft1
Sqle
Lss
Dhcr24
Cyp51a1
Lbr
Dhcr24
Msmo1
Nsdhl
Hsd17b7
Ebp
Dhcr24
Dhcr24
Ebp
Ebp
Sc5d
Dhcr24
Dhcr24
Hmgcr
Soat1
Lipa
ActaCoA
Ac-CoA
Dissipation
Lanol
CoA
H2O
HMG-CoA
CoA
Mva
ATP
Mvn-5P
ADP
ATP
ADP
S-5DPMA
ATP
ADP
Pi
IsopPyr
CO2
Ipe
Gryl-PP
Ppi
FarPP
Ppi
NADPH
Squalen
NADP
Ppi
O2
NADPH
S23O
H2O
NADP
Lastrol
NADPH
2425Di
NADP
O2
NADPH
FF-Mas
Formate
NADP
H2O
NADPH
44Dcdol
NADP
NADPH
44DCBol
NADP
NADPH
O2
4M4CA
NADP
H2O
NADP
3K4Mzol
NADPH
CO2
NADPH
4aMzmol
NADP
ZymolI2
5CDBol
NADPH
5aChlol
NADP
NADPH
Lathost
NADP
7-DHMOL
Accumulation
7DHC
NADPH
NADP
Desmost
NADPH
O2
NADP
H2O
NADPH
NADP
NADPH
NADP
VitD3
Accumulation
8Dhchol
NADPH
NADP
CoA
FA
ChoEst
H2O
FA
H2O
PW000014
PW000054
PW000051
PW000163
PW000054
PW000023
PW000141
PW000145