A B C D E F G H I J K L M

Cholesterol synthesis (branch point only) (debranching) Aldose reductase Sorbitol dehydrogenase
Glycogen and galactose metabolism glycogen (n+1) glucose sorbitol fructose Aromatic amino acid metabolism triiodothyronine (T3) dopaquinone melanin
Purine synthesis Pyrimidine synthesis
phenylacetate
NADPH NADP+ NAD+ NADH thyroxine (T4) (multiple steps)
acetyl-CoA x2 (many cycles) Pi Debranching enzyme
4'

α-1,4 bonds 3'

Aromatic amino acid 5-phosphoribosyl pyrophosphate (PRPP) HCO3-
(branching) Glycogen B6 2' Pi (multiple steps) O2 H2O (multiple steps) Tyrosinase O2 H2O
1 2 3 4 5 6 7 8 9 10
1' Pentose phosphate pathway Phenylalanine Tyrosine decarboxylase B6 Dopamine
Thiolase phosphorylase -5 -4 -3 -2 -1 0 1 2 3 4 Dehydrogenase Transaminase B6
hydroxylase hydroxylase (DOPA decarboxylase) β-hydroxylase Methyltransferase glutamine 2 ATP
(many cycles) Glycogen Glucose-6-phosphatase
10 UDP 4:4 Transferase ATP glutathione (reduced) H2O2 phenyllactate phenylpyruvate phenylalanine tyrosine dihydroxy- dopamine norepinephrine epinephrine Glutamine PRPP
CoA synthase Hexokinase or (in endoplasmic reticulum) glutamine Carbamoyl
9
glycogen (n) 1'
phenylanine amidotransferase (GPAT) glutamate
glycogen (n) glucokinase Glutathione reductase Glutathione peroxidase NADP+ NADPH CO2 phosphate
Glycosyl (4:6) 8 -5 -4 -3 -2 -1 0 1 2 3 4 2' 3' 4'
ADP glutamate α-ketoglutarate (L-DOPA) O2 H 2O
UDP-glucose glutathione (oxidized) 2 H 2O α-ketoglutarate PPi glutamate synthetase II
acetoacetyl-CoA transferase 7 1 Glucose α-1,6-Glucosidase SAM SAH
1' (free) Transaminase B6 (CPSII)
(branching 6
α-1,6 bond conjugated (in lysosomes)
UTP glucose- NADP+ NADPH NADP+ NADPH
1 HMG-CoA synthase
acetyl-CoA enzyme)
1 2 3 4 5
bilirubin PPi UTP 1-phosphate
uridyltransferase
-5 -4 -3 -2 -1 0

Phosphoglucomutase
1 2 3 4 2' 3' 4'
NADP+ NADPH biosynthetic reduction reactions
H2O H +
Tetrahydrobiopterin (BH4)
Dihydrobiopterin
Dihydrobiopterin (BH2)
glutamate

p-hydroxyphenylpyruvate
BH4
Dihydrobiopterin
BH2
homovanillic acid (HVA) vanillylmandelic acid (VMA) VMA
5-phosphoribosylamine
2 ADP Pi
1
CoA glucose-1-phosphate glucose-6-phosphate 6-phosphogluconolactone 6-phosphogluconate NADP+ reductase reductase glycine ATP
Steroid hormone synthesis Glucose-6-phosphate Lactonase
NADPH
other primed glycogenin UDP-galactose epimerase dehydrogenase (G6PD) O2 carbamoyl phosphate
6-Phosphogluconate p-hydroxyphenylpyruvate GAR synthetase
β-hydroxy-β-methylglutaryl-CoA glucuronidation (multiple steps) ADP
(HMG-CoA) Glycolysis and Gluconeogenesis dehydrogenase dioxygenase Aromatic amino acid
reactions CO2 CO2 O2 Tryptophan H2O decarboxylase B6 Serotonin Hydroxyindole Aspartate
UDP-glucose UDP-galactose Pi aspartate
Zona glomerulosa (8 cycles) glucose hydroxylase (DOPA decarboxylase) N-acetyltransferase O-methyltransferase transcarbamoylase
HMG-CoA reductase 2 NADPH glycogenin Lactose synthase fructose 2,6-bisphosphate AMP
UDP Galactose-1-phosphate nucleotides 5-phosphoribosyl pyrophosphate (PRPP) ATP Dihydroneopterin alkapton homogentisate tryptophan 5-hydroxy- serotonin (5-HT) N-acetyl-5-HT melatonin
UDP-glucose (inside Golgi of ADP Phosphoglucose ribulose-5-phosphate (spontaneous)
CoA cholesterol uridyltransferase (GALT) tryptophan glycinamide ribonucleotide Pi
2 NADP+ dehydrogenase mammary cells) Phosphofructokinase-2 isomerase PRPP synthetase triphosphate O2 CO2 acetyl-CoA CoA
UDP-glucuronate UDP ATP Homogentisate (GAR)
Ribose-5- SAM SAH
lactose Fructose bisphosphatase-2 dioxygenase niacin
phosphate formate N10-formyl-THF
mevalonate Cholesterol 2 NADH 2 NAD +
galactose-1-phosphate (Bifunctional enzyme) Monoamine N-carbamoyl aspartate
isomerase NADP +
NADPH GAR transformylase
desmolase Phosphomannose GTP-cyclohydrolase maleylacetoacetate oxidase (MAO)
glycosaminoglycans (GAGs) ADP Hexokinase isomerase sedoheptulose- BH4 BH2 THF
Zona fasciculata and zona reticularis ribose-5-phosphate Ribulose-5- H2O
(multiple 3 ATP glycoproteins Galactokinase mannose mannose-6-phosphate fructose-6-phosphate 7-phosphate Dihydrobiopterin Dihydroorotase
phosphate succinylacetoacetate (spontaneous) Maleylacetoacetate
steps) 17α-hydroxylase glycolipids NADP+ NADPH ATP reductase
17, 20 lyase ATP ADP epimerase isomerase formyl-GAR
3 ADP pregnenolone 17-hydroxypregnenolone dehydroepiandrosterone galactose GTP 5-hydroxyindoleacetic acid
galactitol (FGAR)
(DHEA) Aldose reductase glutamine 6 7 7 5 CO2 (spontaneous) fumarylacetoacetate dihydroorotate
Pi + + Transaldolase + + Transketolase B1
CO2 glutamate ATP 4 3 3 5
3β-hydroxysteroid 3β-hydroxysteroid 3β-hydroxysteroid
Fructose-1,6- Fumarylacetaoacetate ATP
Phosphofructokinase-1 succinylacetone H2O
dehydrogenase dehydrogenase dehydrogenase Glutamine: F6P hydrolase
GAGs glucosamine- 6-phosphate bisphophatase NAD+ Dihydroorotate
isopentenyl pyrophosphate (IPP) B6 ADP erythrose- glyceraldehyde- FGAM synthetase
aminotransferase xylulose-5-phosphate Methionine adenosyltransferase ADP
(C5) x3 4-phosphate (step 2) 3-phosphate (step 1) dehydrogenase
17α-hydroxylase 17, 20 lyase Aromatase
progesterone 17-hydroxyprogesterone androstenedione estrone 6 4 TCA acetoacetate fumarate TCA Pi NADH (in mitochondria)
Coenzyme Q (reduced) Coenzyme Q (oxidized) Transketolase B1 + +
(multiple 17β-hydroxysteroid 3 5 ATP 3 Pi H2O
21-hydroxylase 21-hydroxylase fructose-1,6-bisphosphate glutamine
2 PPi steps) dehydrogenase 17β-HSD (in mitochondrial matrix)
(not in gonad) (not in gonad) methionine S-adenosylmethionine (SAM) orotate
2 farnesyl pyrophosphate (FPP) 11-deoxycorticosterone 11-deoxycortisol
(17β-HSD)
testosterone
Aromatase
estradiol
FAD FADH2
(step 3)
Single-carbon metabolism and sulfur-containing amino acids glutamate 2
(C15) x2 (11-DOC) Mitochondrial glycerol Aldolase PRPP Orotate
11β-hydroxylase Methyltransferases CH3 (used for methylation)
11β-hydroxylase 3-phosphate dehydrogenase (flavoprotein) formiminoglycinamidine ribonucleotide phosphoribosyl-
purine synthesis histidine catabolism pyrimidine synthesis
(FGAM) PPi transferase
Squalene synthase 5α reductase Glycerol
NADPH dihydrotestosterone (DHT) kinase Glycerol-3-phosphate dehydrogenase
corticosterone cortisol ATP
11β-hydroxysteroid + glycerol glycerol dihydroxyacetone glyceraldehyde- THF Homocysteine B12 AIR synthetase
NAD NADH S-adenosylhomocysteine (SAH)
2 PPi NADP+ Aldosterone dehydrogenase 2 (11β-HSD2) 3-phosphate +
phosphate (DHAP) Triosephosphate isomerase 3-phosphate methyltransferase orotidine-5'-mono-
ATP ADP NAD NADH NADPH NADP+ NADPH NADP+ ADP
synthase cortisone NADPH NADP+ NADPH NADP+ formate ATP ADP Pi H 2O S-adenosyl- phosphate (OMP)
11β-HSD1 Pi homocysteine Pi
squalene (C30) aldosterone Fructose-1-P aldolase Triose kinase ADP NAD+ B9 folate dihydrofolate tetrahydrofolate N10-formyl-THF N5,N10-methenyl-THF N5,N10-methylene-THF N5-methyl-THF hydrolase
NADP+ NADPH Fructokinase (aldolase B) ATP Dihydrofolate DHFR (THF) C1-THF Methenyl-THF Methenyl-THF Methylene-THF
fructose fructose-1-phosphate Glyceraldehyde-3- aminoimidazole ribonucleotide OMP decarboxylase
reductase synthase cyclohydrolase reductase reductase homocysteine CO2
phosphate dehydrogenase NADH (AIR) (UMP synthase)
(multiple NADPH (DHFR) NH4+
(in adrenal cortex, some in gonad) (in peripheral tissue) ATP ADP glyceraldehyde adenosine nucleotide salvage
steps) NADH Cystathionine serine
+
NADP Glycine β-synthase B6 AIR carboxylase CO2
1,3-bisphosphoglycerate CO2
Fatty acid synthesis cleavage uridine-5'-monophosphate
serine CoA
lanosterol ADP complex (UMP)
protein glycerol-3-phosphate (multiple steps) Phosphoglycerate cystathionine carboxyaminoimidazole ribonucleotide
modification protein sphingosine sphingolipids NAD+
kinase THF (CAIR)
10 O2 modification ATP Serine hydroxymethyltransferase glutathione
CO2 Bisphosphoglycerate Phosphoglycerate Phosphoserine Phosphoserine ATP
B6 (SHMT) B6 Cystathionine
4 NAD+ 8 NADPH Malonyl-CoA ACP transacylase mutase dehydrogenase transaminase phosphatase ATP aspartate UMP kinase
(multiple malonyl-ACP malonyl-CoA 3-phosphoglycerate 3-phopsphopyruvate 3-phosphoserine serine glycine nucleotides γ-lyase B6 (in mitochondrial matrix)
fatty acyl-ACP α-ketobutyrate succinyl-CoA TCA ADP
steps) Glycerol-3-phosphate Pi SAICAR synthetase
4 NADH 8 NADP +
palmitoyl-CoA CoA ACP Phosphoglycerate NAD+ NADH glutamate α-ketoglutarate H 2O Pi lysine porphyrins ADP
acyltransferase CoA arginine glutathione
mutase α-ketoglutarate Amidinotransferase cysteine H2O
2 CO2 (GPAT) ADP Pi phosphatidylserine
β-ketoacyl-ACP Coenzyme A Pi

OM
B7 Acetyl-CoA Malonyl-CoA Lysine-α- (in mitochondrial matrix) UDP
formate synthase (first cycle only) NADPH ornithine O2 NH + Threonine dehydratase
3 7-dehydrocholesterol CoA
(7 cycles) ACP CO2
carboxylase
ATP

HCO3 -
decarboxylase

CO2
2,3-bisphosphoglycerate
2,3-bisphosphoglycerate
phosphatase
2-phosphoglycerate ceramide

sphingosine NADP +
ketoglutarate
reductase
guanidinoacetate
Cysteine
dioxygenase
4
succinylaminoimidazole carboxamide ribonucleotide
(SAICAR)
ATP Nucleoside
3
β-ketoacyl-ACP Acetyl-CoA ACP Enolase threonine diphosphate
NADPH PPi ACP saccharopine Transaminase B6 ADP
phosphatidic acid Long-chain synthase transacylase H2O kinase
7-Dehydro- NAD+ Guanidinioacetate SAM cysteine β-sulfinylpyruvate pyruvate
NADP+ fatty β-ketoacyl-ACP acetyl-ACP acetyl-CoA GTP GDP CO2 Branched chain amino acid breakdown H2O
cholesterol H2O AMP Saccharopine sulfinate Adenylosuccinate lyase

.C
acyl-CoA α-ketoglutarate NADH methyltransferase glutamate 2-
sulfite (SO3 )
reductase CO2 CoA ACP alanine dehydrogenase SAH Pi α-ketoglutarate
synthetase NADPH oxaloacetate phosphoenolpyruvate (GAMT) ADP fumarate
Pi ATP β-ketoacyl-ACP Phosphoenolpyruvate glutamate ATP UTP
cholesterol phospholipids glutamate phosphocreatine Cysteine sulfinite
reductase carboxykinase Pyruvate ADP (spontaneous) Sulfite
Triacylglycerol NADP+ leucine isoleucine valine α-aminoadipic semialdehyde decarboxylase CO2 ATP

DD
NADH kinase Alanine B6 Creatine oxidase aminoiminidazole carboxamide ribonucleotide
synthetase 1,2-diacylglycerol Malate NAD+ Hypotaurine CTP synthetase
CoA β-hydroxyacyl-ACP Fatty acid synthase Pi glutamate dehydrogenase ATP transaminase (ALT) creatine kinase creatinine (AICAR)
complex + α-ketoglutarate α-ketoglutarate α-ketoglutarate Dehydrogenase dehydrogenase ADP + Pi
ADP NAD NADP+ NADPH CO2 H 2O (excreted) hypotaurine taurine NH4+
fatty acyl-CoA Aspartate Transaminase Transaminase Transaminase NADH bile salts sulfate (SO42-)
palmitate (C16) β-hydroxyacyl-ACP Lactate dehydrogenase N10-formyl-THF
Citrate lyase amino- pyruvate lactate B6 B6 B6 α-aminoadipate glutamine
vitamin D bile salts cell lipoproteins dehydratase H2O transferase malate glutamate glutamate glutamate NAD+ NADH (spontaneous)
ATP CoA Malic enzyme (spontaneous) AICAR transformylase
(skin) (liver) membranes CoA B6 (AST) α-ketoglutarate
NAD+

LA
NADH THF
trans-enoyl-ACP B6 glutamate
α-ketoglutarate Transaminase
triacylglycerol citrate aspartate α-ketoisocaproate α-keto-β-methylvalerate α-ketoisovalerate glutamate Urea cycle
Enoyl-ACP NADPH oxaloacetate formaminoimidazole carboxamide ribonucleotide
α-ketoadipate CTP
reductase + -
(FAICAR)
NH4 HCO
NADP+ tricarboxylate glutamate/ dicarboxylate mitochondrial pyruvate 3

FI
transporter aspartate transporter transporter carrier glutamate
Fatty acid oxidation Argininosuccinate Transaminase
(n+2) fatty acyl-ACP Citric acid cycle (tricarboxylic acid cycle) citrulline
IMP cyclohydrolase H2O
α-ketoisocaproate α-keto-β-methylvalerate α-ketoisovalerate α-ketoadipate synthetase B6
α-ketoglutarate
lipoproteins 2 ATP ornithine/citrulline
HCO3- PPi
(extrahepatic metabolism) free fatty acids free fatty acids aspartate α-ketoglutarate NAD+ CoA Branched-chain NAD+ CoA NAD+ CoA NAD+ CoA Carbamoyl transporter
CoA ATP AMP PPi pyruvate ATP


citrate ketoacid B1 BCKAD B1 BCKAD B1 α-ketoadipate B1 phosphate 2 ADP aspartate
AMP inosine monophosphate
Diet fatty acids fatty acids fatty acids fatty acyl- CoA CoA Pyruvate B7 NADH CO2 dehydrogenase NADH CO2 NADH CO2 dehydrogenase synthetase I NO (IMP)
> C24 to peroxisomes ≤ C12 > C14 and < C22 Long-chain fatty carboxylase CO2 (BCKAD) NADH (CPS1) citrulline Nitric oxide glutamine NH4+ NH4+
Legend
carnitine
acyl-CoA synthetase
lysine
Acyl-CoA
Synthetase CPT1
AST
B6
ADP
ATP NAD+
Pyruvate B1 isovaleryl-CoA α-methylbutyryl-CoA isobutyryl-CoA glutaryl-CoA
CO2 Pi
Ornithine Pi
synthase Asparagine synthetase
glutamate
AMP deaminase Adenylosuccinate
synthetase
GTP
H2O 4
NADH dehydrogenase transcarbamoylase (OTC) NADP+ GDP NAD+
Carbohydrates Amino acids Pi NAD+
Isovaleryl-CoA carbamoyl H2O Pi IMP dehydrogenase
CoA (multiple steps) FAD argininosuccinate
carnitine fatty acyl-carnitine dehydrogenase Glutaryl-CoA phosphate NH4+ ATP NADH
carnitine transporter glutamate oxaloacetate acetyl-CoA NADPH aspartate NADP+
NADH dehydrogenase Asparaginase
Oxidative ethanol (spontaneous) FADH2 polyamines ornithine O2 GMP reductase
Nucleotides NAD+ H 2O AMP NADPH
metabolism Alcohol CPT2 Translocase
Base salvage
β-methylcrotonyl-CoA propionyl-CoA odd-chain fatty oxaloacetate Argininosuccinate

Lipids Heme
metabolism
4 dehydrogenase

acetaldehyde
NADH
fatty acids ≤ C12
CoA ATP AMP PPi
fatty acyl-CoA CoA
acetyl-CoA
ATP
HCO3-
ATP
HCO3-
(last three carbons only) acyl-CoA
crotonyl-CoA

H2O Hydratase
Ornithine aminotransferase B6
ornithine/citrulline
transporter
lyase

fumarate asparagine
PPi
adenylosuccinate XMP
Fatty acyl-CoA synthetase β-methylcrotonyl-CoA Propionyl-CoA aspartate arginine
ornithine Arginase PPi Hypoxanthine-guanine
(long chains only) carboxylase B7 carboxylase B7 ATP ADP Pi glutamine
Multiple steps FAD ADP ADP Adenylosuccinate phosphoribosyltransferase ATP
black dashed arrow Acyl-CoA citrate β-hydroxybutyryl CoA urea H2O (HGPRT)
Ketone body metabolism TCA Citrate H2O CoA glutamate glutamic semialdehyde lyase PRPP GMP synthetase
dehydrogenases β-methylglutaconyl-CoA methylmalonyl-CoA
Participates in additional reactions FADH2 oxaloacetate synthase H2O glutamate AMP + PPi
gray dashed arrow acetaldehyde NAD+ NADPH NADP+
NADH Aconitase fumarate
β-methylglutaconyl-CoA Methylmalonyl-CoA Dehydrogenase (spontaneous) hypoxanthine
Acetaldehyde NAD+ trans-enoyl-CoA Pyrroline-5- NADPH NADP+
Multiple cycles NAD + cis-aconitate hydratase mutase B12 NADH
heavy gray arrow dehydrogenase acetyl-CoA x2 carboxylate synthetase
Malate H2O pyrroline- pyrroline-5-carboxylate proline Adenine
NADH H2O succinyl-CoA phosphoribosyltransferase
thin gray arrow
Crosses membrane Hydratase dehydrogenase º-hydroxy-β-methylglutaryl-CoA
succinyl-CoA
acetoacetyl-CoA 5-carboxylate Pyrroline-5-carboxylate HGPRT
Aconitase (HMG-CoA) AMP adenine guanine GMP
acetate reductase
Thiolase (many cycles) CoA
Outer mitochondrial membrane CoA CoA Thiolase PPi PRPP PRPP PPi
β-hydroxyacyl-CoA Methylmalonyl-CoA mutase B12 isocitrate HMG-CoA lyase
Acetyl-CoA ATP ATP ATP
Inner mitochondrial membrane acetoacetyl-CoA
malate Nucleotide breakdown Deoxyribonucleotides Adenylate AMP GMP kinase
synthetase NAD +
Isocitrate NAD +
acetoacetate acetyl-CoA TCA acetyl-CoA x2 RNA/DNA kinase
AMP Hydroxyacyl-CoA methylmalonyl-CoA ADP PRPP synthetase ADP
Plasma membrane PPi dehydrogenases dehydrogenase Nucleases ATP GTP CTP UTP ATP
acetyl-CoA NADH NADH Ammonium capture and release B6
ADP
Vitamins 5 acetyl-CoA CoA
HMG-CoA synthase (in liver)
β-ketoacyl-CoA
Propionyl-CoA carboxylase B7

ATP H2O Fumarase

CO2
NH 4
+
H 2O
Glutaminase
NH4+ H2O
(deoxy) GMP (deoxy) AMP (deoxy) CMP UMP deoxyTMP
H 2O
Phosphatase
H 2O
Phosphatase
H 2O
Phosphatase
H 2O
Phosphatase
ADP
ribose-5-P
GDP 5
Enzyme cofactor: CoA Pi Pi Pi Pi Phosphopentomutase
α-ketoglutarate glutamate glutamine H2O H2O
B1 thiamine α-ketoacid dehydrogenases and
β-hydroxy-β-methyl-glutaryl-CoA
Thiolase HCO3 -

Nucleotidase
H2O
Nucleotidase
H2O
Nucleotidase
H2O
Nucleotidase Nucleotidase Nucleoside GTP
uracil Pi
ATP Nucleoside
transketolase NADH NAD +
(HMG-CoA) (odd-chain fatty acids only) NH4+ H2O ATP ADP Pi Pi Pi Pi ADP GDP CDP UDP ribose-1-P uridine
Precursor: propionyl-CoA Glutamate Pi Pi H2O diphosphate Uridine phosphorylase diphosphate
B2 riboflavin FAD/FADH2 (n-2) fatty acyl-CoA acetyl-CoA fumarate α-ketoglutarate CoA dehydrogenase-1 Glutamine synthetase AMP kinase GDP ADP kinase
TCA HMG-CoA lyase (in liver) dehydrogenase 1 B NAD+ deaminase +
N-acetylglutamate synthase NH4 NH4 +
B3 niacin
Precursor:
NAD+/NADH and NADP+/NADPH cholesterol (cytosol) CO2 many many acetyl-CoA CoA
(deoxy) guanosine
CO2 amino Transaminases B6 α-ketoacids (deoxy) IMP (deoxy) (deoxy) thymidine
B5 pantothenate Precursor: CoA
acetyl-CoA acetoacetate acetone
NADH acids
Glutamate N-acetylglutamate
Purine
nucleoside
Pi
H2O
cytidine Pyrimidine
nucleoside
uridine dADP dGDP dCDP dUDP ATP S-adenosyl methionine biopterin GTP
(spontaneous) Thymidine Pi
Enzyme cofactor: decarboxylase CO2 phosphorylase (deoxy) Nucleotidase deaminase Coenzyme A
pyridoxal heme b ATP ATP ATP ATP
B6 phosphate
transaminases and other reactions
NADH TCA 2 H+ succinyl-CoA
glutamate α-ketoglutarate
acetate H2O
Hydrolase urea cycle (regulation)
ribose-1-P
Adenosine
Pi phosphorylase
deoxy Kinase Kinase Kinase Kinase
involving amino acids β-hydroxybutyrate Uridine Pi ribose-1-P NADH, FADH2
Heme synthesis Ferrochelatase Pi guanine deaminase ADP ADP ADP ADP
dehydrogenase Succinyl-CoA (deoxy) (deoxy)
succinic semialdehyde γ-aminobutyric 3 H+ phosphorylase
B7 biotin Enzyme cofactor: carboxylases
glycine succinyl-CoA
NAD+ Fe2+
protoporphyrin IX
synthetase GDP
NAD+ GABA acid (GABA)
adenosine
H2O NH4+
inosine (deoxy)
ribose-1-P
Succinic semialdehyde aminotransferase ADP + Pi ATP (spontaneous) Nucleoside salvage
GTP dATP dGTP dCTP dUTP
NAD +
NADH succinyl-CoA succinate CoA dehydrogenase B6
Pi ATP ADP
uracil thymine
B9 folate Single-carbon carrier CoA δ-aminolevulinic 3 H2O2
Protoporphyrinogen 4 H+ CoA
NADH Purine nucleoside
phosphorylase NH4+ adenosine adenosine 5’-monophosphate (AMP)
acid synthase B6
β-hydroxybutyrate acetoacetate acetoacetyl-CoA acetyl-CoA x2 NADH NAD + Oxidative phosphorylation V (deoxy) NADPH NADPH
CO2 Succinyl-CoA:acetoacetate Thiolase IX oxidase succinate 4 H+ 2 H+ Pi ADP ATP ribose-1-P Dihydropyrimidine H 2O Adenosine kinase
Enzyme cofactor: β-hydroxybutyrate 3 O2 dUTP
B12 cobalamin methylmalonyl-CoA mutase and dehydrogenase transferase (SCOT)
(in extrahepatic tissues)
1
/2 O2 + 2H +
H2O hypoxanthine NADP+
dehydrogenase
(DPD)
NADP+
Phosphatase
Pi
pyrophosphatase
PPi ATP ADP
homocysteine methyltransferase δ-aminolevulinic acid (δ-ALA) protoporphyrinogen IX I
FADH2 II FAD Thymidylate cytidine cytidine 5’-monophosphate (CMP)
- - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - -III - - - -- - - - - - - - - - - - - - - - - - - - - - - - - - - - - - H 2O H 2O NH4+ synthase ATP ADP Uridine-cytidine kinase
I 2e 2e 2e IV Guanine dihydrouracil dihydrothymine uridine uridine 5’-monophosphate (UMP)
phosphate adenine nucleotide Xanthine (TS)
6 protoporphyrinogen IX
+ + + + + + + + +
2 e-
+ + + + + + +
CoQ
+ + + + + + + + + + + + + + + +
Cyt c
+ + + + + + + + + + + + + + + + +
transporter

+ + + +
transporter

+ + +
NH4+
deaminase oxidase
O2
Dihydropryimidinase
dCMP
Deoxynucleotide
deaminase
dUMP dTMP
Kinase
dTDP
ATP ADP 6
NADH Succinate Cytochrome c Cytochrome c ATP synthase H2O2 ATP ADP
2 CO2 2 H2O 4H + dehydrogenase dehydrogenase reductase oxidase (Complex V) β-ureidopropionate β-ureidoisobutyrate thioredoxin
Pathways of xanthine 5 10
N ,N - ATP
(Complex III) 4 H+ (Complex IV) 2 H+ Pi ADP ATP (reduced) dihydrofolate deoxycytidine deoxycytidine 5’-monophosphate (dCMP)
(Complex I) (Complex II) 3 H+ methylene-THF Kinase
O2 Deoxycytidine kinase

Human Metabolism δ-ALA x2

2 H2O

porphobilinogen (PBG) x4
H2O

hydroxymethylbilane
H2O

uroporphyrinogen III
4 CO2

coproporphyrinogen III
Coproporphyrinogen oxidase
Heme degradation
3 NADPH 3 NADP+ NADPH NADP+ UDP-glucuronate x2 UDP x2 feces
O2

Xanthine oxidase
H2O

H2O2 NH4
CO2

+
Ureidopropionase CO2

NH +
Thioredoxin
reductase
NADP+

NADPH Ribonucleotide
glycine
SHMT DHFR
NADPH
ADP
ATP ADP
VERSION 10.18 4
reductase THF thymidine deoxythymidine-5'-monophosphate (dTMP)
δ-ALA dehydratase PBG deaminase Uroporphyrinogen III Uroporphyrinogen heme b biliverdin bilirubin conjugated bilirubin urine Thymidine kinase
4 NH4+ Biliverdin reductase UDP-glucuronyltransferase thioredoxin serine NADP+
Email questions or comments to: metabolicpathways@stanford.edu synthase decarboxylase β-alanine β-aminoisobutyric dTTP deoxyuridine deoxyuridine 5’-monophosphate (dUMP)
3 O2 Fe 3+ CO bile uric acid (oxidized)
acid
Copyright 2017 Stanford School of Medicine. All rights reserved. Heme oxygenase ATP ADP
This work is licensed under a Creative Commons Attribution-
NonCommercial-NoDerivatives 4.0 International License.

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H I J K L M