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 Dehydrogenase Transaminase B6
hydroxylase hydroxylase (DOPA decarboxylase) β-hydroxylase Methyltransferase glutamine 2 ATP
(many cycles) Glycogen -5 -4 -3 -2 -1 0 1 2 3 4
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 H2O
UDP-glucose glutathione (oxidized) 2 H2O α-ketoglutarate PPi glutamate synthetase II
acetoacetyl-CoA transferase 7 1 Glucose α-1,6-Glucosidase SAM SAH
Transaminase B6 (CPSII)
1' (free) (in lysosomes)

1 1
(branching 6
α-1,6 bond conjugated UTP glucose- NADP+ NADPH glutamate NADP+ NADPH
acetyl-CoA 1 2 3 4 5 NADP+ NADPH biosynthetic reduction reactions homovanillic acid (HVA) vanillylmandelic acid (VMA) VMA 2 ADP Pi
HMG-CoA synthase enzyme) bilirubin PPi UTP 1-phosphate
-5 -4 -3 -2 -1 0 1 2 3 4 2' 3' 4'
H2O H +
Tetrahydrobiopterin (BH4) Dihydrobiopterin (BH2) BH4 BH2 5-phosphoribosylamine
uridyltransferase Phosphoglucomutase Dihydrobiopterin p-hydroxyphenylpyruvate Dihydrobiopterin
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
glutamate Pi + + Transaldolase + + Transketolase B1
CO2 3β-hydroxysteroid 3β-hydroxysteroid ATP 4 3 3 5
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)

2 2
(not in gonad) (not in gonad) methionine S-adenosylmethionine (SAM) orotate
(17β-HSD) (step 3)
Aromatase FAD FADH2 Single-carbon metabolism and sulfur-containing amino acids glutamate
farnesyl pyrophosphate (FPP) 11-deoxycorticosterone 11-deoxycortisol testosterone estradiol
(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 H2O 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 (UMP synthase)
(multiple NADPH (DHFR) NH4+ (AIR)
(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 nucleotides ATP aspartate UMP kinase
(multiple malonyl-ACP malonyl-CoA 3-phosphoglycerate 3-phopsphopyruvate 3-phosphoserine serine glycine 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 H2O Pi lysine porphyrins ADP
acyltransferase CoA arginine glutathione
mutase α-ketoglutarate Amidinotransferase cysteine H2O
2 CO2 (GPAT) ADP Pi phosphatidylserine
β-ketoacyl-ACP B7 Acetyl-CoA Malonyl-CoA (in mitochondrial matrix) Coenzyme A Pi UDP
Lysine-α-

3 3
formate synthase (first cycle only) NADPH ornithine O2 NH4 + Threonine dehydratase
carboxylase decarboxylase 2,3-bisphosphoglycerate 2-phosphoglycerate ceramide ketoglutarate
ATP 2,3-bisphosphoglycerate Cysteine
CoA reductase succinylaminoimidazole carboxamide ribonucleotide ATP
7-dehydrocholesterol (7 cycles) ACP CO2 dioxygenase Nucleoside
phosphatase sphingosine NADP +
guanidinoacetate (SAICAR)
HCO3 -
CO2 threonine diphosphate
NADPH PPi ACP β-ketoacyl-ACP Acetyl-CoA ACP Enolase saccharopine B6
phosphatidic acid Long-chain H2O Transaminase ADP kinase
7-Dehydro- synthase transacylase NAD+ cysteine β-sulfinylpyruvate pyruvate
fatty acetyl-ACP acetyl-CoA Guanidinioacetate SAM
cholesterol NADP+ H2O AMP
β-ketoacyl-ACP GTP GDP CO2 Branched chain amino acid breakdown Saccharopine sulfinate H2O Adenylosuccinate lyase
acyl-CoA α-ketoglutarate NADH methyltransferase glutamate sulfite (SO3 )
2-
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
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 H2O (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
NADH NAD+ 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+
HCO3 -
NADP+ tricarboxylate glutamate/ dicarboxylate mitochondrial pyruvate
transporter aspartate transporter transporter carrier glutamate
(n+2) fatty acyl-ACP Fatty acid oxidation Citric acid cycle (tricarboxylic acid cycle) Argininosuccinate Transaminase
IMP cyclohydrolase
α-ketoisocaproate α-keto-β-methylvalerate α-ketoisovalerate α-ketoadipate citrulline synthetase B6 H2O
lipoproteins α-ketoglutarate
HCO3- 2 ATP ornithine/citrulline PPi
(extrahepatic metabolism) free fatty acids free fatty acids aspartate NAD+ CoA Branched-chain NAD+ CoA NAD+ CoA NAD+ CoA Carbamoyl transporter
CoA ATP AMP PPi
α-ketoglutarate
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)

4
> C14 and < C22 Long-chain fatty carboxylase (BCKAD) NH4+
Legend > C24 to peroxisomes ≤ C12
acyl-CoA synthetase AST
B6 ATP NAD+
CO2
Pyruvate B1
NADH CO2 (CPS1) Pi citrulline Nitric oxide
synthase
glutamine
Asparagine synthetase
NH4+
AMP deaminase Adenylosuccinate H2O
carnitine Acyl-CoA
CPT1 isovaleryl-CoA α-methylbutyryl-CoA isobutyryl-CoA glutaryl-CoA Ornithine Pi GTP
lysine Synthetase ADP dehydrogenase glutamate synthetase
NADH 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 H2O AMP NADPH
metabolism Alcohol NAD+ CPT2 Translocase
Base salvage
4
β-methylcrotonyl-CoA propionyl-CoA odd-chain fatty oxaloacetate Argininosuccinate
dehydrogenase acetyl-CoA (last three carbons only) crotonyl-CoA
Heme NADH CoA ATP AMP PPi acyl-CoA ornithine/citrulline lyase PPi
Lipids HCO3- HCO3- Ornithine aminotransferase B6 transporter adenylosuccinate XMP
metabolism acetaldehyde fatty acids ≤ C12 fatty acyl-CoA CoA ATP
β-methylcrotonyl-CoA
ATP
Propionyl-CoA
H2O Hydratase aspartate arginine fumarate asparagine
Fatty acyl-CoA synthetase 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
Ketone body metabolism TCA Citrate H2O CoA glutamate glutamic semialdehyde urea H2O lyase PRPP (HGPRT) GMP synthetase
dehydrogenases β-methylglutaconyl-CoA methylmalonyl-CoA
Participates in additional reactions FADH2 oxaloacetate synthase H2O glutamate AMP + PPi
acetaldehyde NAD+ NADPH NADP+
gray dashed arrow
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 phosphoribosyltransferase
thin gray arrow
Crosses membrane Hydratase
succinyl-CoA 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
5 5
ADP B6
HMG-CoA synthase (in liver) CO2 Glutaminase ribose-5-P
Propionyl-CoA carboxylase B7 H2O H2O H2O H2O
Vitamins acetyl-CoA CoA β-ketoacyl-CoA H2O Fumarase NH4 +
H2O NH4+ H2O
Phosphatase Phosphatase Phosphatase Phosphatase
ADP GDP
ATP (deoxy) GMP (deoxy) AMP (deoxy) CMP UMP deoxyTMP
Enzyme cofactor: Pi Pi Pi Pi Phosphopentomutase
B1 thiamine α-ketoacid dehydrogenases and
β-hydroxy-β-methyl-glutaryl-CoA
Thiolase CoA
HCO3 -
α-ketoglutarate glutamate glutamine
Nucleotidase
H2O
Nucleotidase
H2O
Nucleotidase
H2O
Nucleotidase
H2O
Nucleotidase
H2O
Nucleoside GTP
uracil Pi
ATP Nucleoside
transketolase
(odd-chain fatty acids only) NADH NAD +
NH4+ H2O ATP ADP Pi ribose-1-P uridine
(HMG-CoA) Glutamate Pi Pi Pi Pi Pi ADP GDP CDP UDP diphosphate diphosphate
H2O
B2 riboflavin
Precursor:
FAD/FADH2 (n-2) fatty acyl-CoA acetyl-CoA
propionyl-CoA
fumarate α-ketoglutarate CoA dehydrogenase-1 Glutamine synthetase AMP kinase GDP
Uridine phosphorylase
ADP kinase
TCA HMG-CoA lyase (in liver) dehydrogenase 1 B NAD+ deaminase
N-acetylglutamate synthase NH4 +
NH4
B3
+
Precursor:
niacin cholesterol (cytosol) many many (deoxy) guanosine
NAD+/NADH and NADP+/NADPH CO2 CO2 acetyl-CoA CoA (deoxy) IMP (deoxy) (deoxy) thymidine
amino Transaminases B6 α-ketoacids
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
heme b
B6 pyridoxal
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
ATP
Kinase
ATP
Kinase
ATP
Kinase
ATP
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)
B7 biotin Enzyme cofactor: carboxylases
glycine succinyl-CoA
NAD+ Fe2+
protoporphyrin IX
synthetase GDP
NAD+
succinic semialdehyde
GABA
γ-aminobutyric
acid (GABA)
3 H+ adenosine
H2O NH4+
inosine
phosphorylase
(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
B9 folate Single-carbon carrier CoA δ-aminolevulinic 3 H2O2
Protoporphyrinogen 4 H+ CoA
NADH Purine nucleoside uracil thymine
NH4+ adenosine adenosine 5’-monophosphate (AMP)
acid synthase B6
β-hydroxybutyrate acetoacetate acetoacetyl-CoA acetyl-CoA x2 NADH NAD + Oxidative phosphorylation V phosphorylase (deoxy) NADPH NADPH
CO2 Succinyl-CoA:acetoacetate Thiolase IX oxidase succinate 4 H+ 2 H+ Pi ADP ATP ribose-1-P Dihydropyrimidine H2O Adenosine kinase
Enzyme cofactor: β-hydroxybutyrate 3 O2
B12 cobalamin methylmalonyl-CoA mutase and dehydrogenase transferase (SCOT)
(in extrahepatic tissues)
/2 O2 + 2H
1 +
H2O hypoxanthine NADP+
dehydrogenase
(DPD)
NADP+
Phosphatase
Pi
dUTP
pyrophosphatase
PPi ATP ADP
homocysteine methyltransferase δ-aminolevulinic acid (δ-ALA) protoporphyrinogen IX I
FADH2 II FAD Thymidylate cytidine cytidine 5’-monophosphate (CMP)
- - - - - - - - - - - - - - - - - - - - - - - - - - - - - - -III - - - -- - - - - - - - - - - - - - - - - - - - - - - - - - - - - H2O H2O NH4+ synthase ATP ADP Uridine-cytidine kinase
2e -
2e 2e -
IV Guanine dihydrouracil dihydrothymine uridine uridine 5’-monophosphate (UMP)

6 6
I phosphate adenine nucleotide Xanthine (TS)
CoQ Cyt c transporter transporter deaminase O2 dTMP dTDP
oxidase dCMP dUMP
2 e- NH4+ Dihydropryimidinase Deoxynucleotide Kinase ATP ADP
+ + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + +
protoporphyrinogen IX deaminase
NADH Succinate Cytochrome c Cytochrome c ATP synthase H2O2 ATP ADP

Pathways of
2 CO2 2 H2O 4H + dehydrogenase dehydrogenase reductase oxidase (Complex V) xanthine β-ureidopropionate β-ureidoisobutyrate thioredoxin N ,N -
5 10
Pi ADP ATP (reduced) dihydrofolate ATP deoxycytidine deoxycytidine 5’-monophosphate (dCMP)
(Complex I) (Complex II) (Complex III) 4 H+ (Complex IV) 2 H+ 3 H+ methylene-THF Kinase Deoxycytidine kinase
O2

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

NH4
Thioredoxin
reductase
NADP+

NADPH Ribonucleotide
glycine
SHMT DHFR
NADPH
ADP
ATP ADP
VERSION 10.18
+ +

heme b biliverdin bilirubin conjugated bilirubin reductase THF thymidine deoxythymidine-5'-monophosphate (dTMP)
δ-ALA dehydratase PBG deaminase Uroporphyrinogen III Uroporphyrinogen 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.

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