A B C D E F G H I J K L M Aldose reductase Sorbitol dehydrogenase Cholesterol synthesis Glycogen and galactose metabolism (branch point only) (debranching) glucose sorbitol fructose Aromatic amino acid metabolism Purine synthesis Pyrimidine synthesis glycogen (n+1) phenylacetate triiodothyronine (T3) dopaquinone melanin + + thyroxine (T4) (multiple steps) (many cycles) P 4' NADPH NADP NAD NADH acetyl-CoA x2 i Debranching enzyme 3' - α-1,4 bonds Aromatic amino acid 5-phosphoribosyl pyrophosphate (PRPP) HCO B 2' P 3 (branching) Glycogen 6 i (multiple steps) O H O (multiple steps) Tyrosinase O H O 1' 2 2 2 2 B 1 2 3 4 5 6 7 8 9 10 Pentose phosphate pathway Phenylalanine Tyrosine decarboxylase 6 Dopamine B Thiolase phosphorylase -5 -4 -3 -2 -1 0 1 2 3 4 Transaminase 6 glutamine 2 ATP (many cycles) Glycogen Glucose-6-phosphatase Dehydrogenase hydroxylase hydroxylase (DOPA decarboxylase) β-hydroxylase Methyltransferase 10 UDP 4:4 Transferase ATP glutathione (reduced) H O phenyllactate phenylpyruvate phenylalanine tyrosine dihydroxy- dopamine Glutamine PRPP CoA synthase Hexokinase or (in endoplasmic reticulum) 2 2 norepinephrine epinephrine glutamine Carbamoyl 9 1' phenylanine amidotransferase (GPAT) glutamate glycogen (n) Glutathione reductase Glutathione peroxidase + phosphate glycogen (n) -5 -4 -3 -2 -1 0 1 2 3 4 2' 3' 4' glucokinase 8 NADP NADPH glutamate α-ketoglutarate CO2 O H O Glycosyl (4:6) UDP-glucose ADP (L-DOPA) 2 2 synthetase II glutathione (oxidized) 2 H O α-ketoglutarate PPi glutamate acetoacetyl-CoA 7 1 Glucose -1,6-Glucosidase 2 SAM SAH transferase α B (CPSII) 1' Transaminase 6 6 (free) (in lysosomes) + + (branching α-1,6 bond conjugated UTP glucose- + NADP NADPH NADP NADPH 1 2 3 4 5 NADP NADPH biosynthetic reduction reactions glutamate acetyl-CoA -5 -4 -3 -2 -1 0 1 2 3 4 2' 3' 4' H O H+ homovanillic acid (HVA) vanillylmandelic acid (VMA) VMA 2 ADP P HMG-CoA synthase enzyme) bilirubin PP UTP 1-phosphate 2 i 1 i Tetrahydrobiopterin (BH4) Dihydrobiopterin (BH2) BH4 BH2 5-phosphoribosylamine 1 uridyltransferase Phosphoglucomutase Dihydrobiopterin p-hydroxyphenylpyruvate Dihydrobiopterin 6-phosphogluconolactone 6-phosphogluconate + CoA glucose-1-phosphate glucose-6-phosphate NADP glycine Steroid hormone synthesis Glucose-6-phosphate Lactonase reductase reductase ATP other NADPH O carbamoyl phosphate primed glycogenin UDP-galactose epimerase dehydrogenase (G6PD) 6-Phosphogluconate 2 p-hydroxyphenylpyruvate GAR synthetase β-hydroxy-β-methylglutaryl-CoA glucuronidation (multiple steps) ADP Glycolysis and Gluconeogenesis dehydrogenase dioxygenase Aromatic amino acid (HMG-CoA) reactions CO O H O Aspartate CO 2 2 Tryptophan 2 decarboxylase B6 Serotonin Hydroxyindole UDP-glucose UDP-galactose 2 P aspartate Zona glomerulosa (8 cycles) glucose hydroxylase (DOPA decarboxylase) N-acetyltransferase O-methyltransferase i transcarbamoylase HMG-CoA reductase 2 NADPH glycogenin Lactose synthase fructose 2,6-bisphosphate AMP Galactose-1-phosphate alkapton homogentisate N-acetyl-5-HT melatonin UDP-glucose UDP (inside Golgi of nucleotides 5-phosphoribosyl pyrophosphate (PRPP) ATP ribulose-5-phosphate Dihydroneopterin tryptophan 5-hydroxy- serotonin (5-HT) cholesterol uridyltransferase (GALT) ADP Phosphoglucose (spontaneous) glycinamide ribonucleotide P CoA + dehydrogenase triphosphate tryptophan i 2 NADP UDP mammary cells) Phosphofructokinase-2 ATP isomerase PRPP synthetase O Homogentisate CO acetyl-CoA CoA (GAR) UDP-glucuronate Ribose-5- 2 2 lactose Fructose bisphosphatase-2 dioxygenase SAM SAH phosphate niacin Cholesterol 2 NADH 2 NAD+ (Bifunctional enzyme) formate N10-formyl-THF N-carbamoyl aspartate mevalonate galactose-1-phosphate isomerase NADP+ NADPH Monoamine GAR transformylase desmolase Phosphomannose GTP-cyclohydrolase maleylacetoacetate oxidase (MAO) glycosaminoglycans (GAGs) ADP sedoheptulose- BH BH THF Zona fasciculata and zona reticularis Hexokinase isomerase ribose-5-phosphate Ribulose-5- H O 4 2 (multiple 3 ATP glycoproteins mannose mannose-6-phosphate fructose-6-phosphate 2 Dihydroorotase Galactokinase 7-phosphate phosphate Maleylacetoacetate Dihydrobiopterin steps) glycolipids NADP+ NADPH ATP succinylacetoacetate (spontaneous) 17α-hydroxylase 17, 20 lyase epimerase isomerase reductase ATP ADP GTP formyl-GAR 3 ADP pregnenolone 17-hydroxypregnenolone dehydroepiandrosterone galactitol galactose 5-hydroxyindoleacetic acid glutamine 6 7 7 5 (spontaneous) (FGAR) dihydroorotate (DHEA) Aldose reductase B CO fumarylacetoacetate P + + Transaldolase + + Transketolase 1 2 CO glutamate ATP i 2 3β-hydroxysteroid 3 -hydroxysteroid 3β-hydroxysteroid 4 3 3 5 β Fructose-1,6- H O Fumarylacetaoacetate ATP dehydrogenase dehydrogenase dehydrogenase Phosphofructokinase-1 succinylacetone 2 Glutamine: F6P bisphophatase hydrolase NAD+ isopentenyl pyrophosphate (IPP) GAGs glucosamine- 6-phosphate B ADP glyceraldehyde- FGAM synthetase Dihydroorotate aminotransferase 6 erythrose- ADP (step 2) (step 1) xylulose-5-phosphate Methionine adenosyltransferase dehydrogenase (C5) x3 17α-hydroxylase 17, 20 lyase Aromatase 4-phosphate 3-phosphate progesterone 17-hydroxyprogesterone androstenedione 6 4 TCA acetoacetate fumarate TCA Pi NADH (in mitochondria) estrone B Coenzyme Q Coenzyme Q Transketolase 1 + + 17β-hydroxysteroid (reduced) (oxidized) 3 5 ATP 3 P H O (multiple 21-hydroxylase 21-hydroxylase fructose-1,6-bisphosphate i 2 2 PP dehydrogenase 17β-HSD glutamine i steps) (not in gonad) (not in gonad) (in mitochondrial matrix) orotate (17β-HSD) (step 3) methionine S-adenosylmethionine (SAM) FAD FADH Aromatase 2 Single-carbon metabolism and sulfur-containing amino acids glutamate 2 farnesyl pyrophosphate (FPP) 11-deoxycorticosterone 11-deoxycortisol testosterone estradiol 2 (11-DOC) PRPP Orotate (C15) x2 11 -hydroxylase Mitochondrial glycerol Aldolase Methyltransferases β CH (used for methylation) phosphoribosyl- 11β-hydroxylase 3-phosphate dehydrogenase (avoprotein) purine synthesis histidine catabolism pyrimidine synthesis 3 formiminoglycinamidine ribonucleotide (FGAM) PP transferase 5 reductase Glycerol i Squalene synthase NADPH α corticosterone cortisol dihydrotestosterone (DHT) kinase Glycerol-3-phosphate dehydrogenase ATP + glycerol glycerol dihydroxyacetone glyceraldehyde- Homocysteine B 11β-hydroxysteroid NAD NADH THF 12 S-adenosylhomocysteine (SAH) AIR synthetase + 3-phosphate 2 PP NADP Aldosterone dehydrogenase 2 (11β-HSD2) 3-phosphate + phosphate (DHAP) Triosephosphate isomerase methyltransferase orotidine-5'-mono- i ATP ADP NAD NADH + synthase NADPH NADP+ NADPH NADP+ formate ATP ADP H O NADPH NADP NADPH NADP+ ADP cortisone Pi 2 S-adenosyl- phosphate (OMP) 10 5 10 5 10 5 homocysteine + B Pi 11β-HSD1 Fructose-1-P aldolase Triose kinase ADP Pi 9 folate dihydrofolate tetrahydrofolate N -formyl-THF N ,N -methenyl-THF N ,N -methylene-THF N -methyl-THF squalene (C30) aldosterone + NAD hydrolase NADP NADPH Fructokinase (aldolase B) Dihydrofolate DHFR (THF) C1-THF Methenyl-THF Methenyl-THF Methylene-THF ATP Glyceraldehyde-3- OMP decarboxylase fructose fructose-1-phosphate reductase synthase cyclohydrolase reductase reductase aminoimidazole ribonucleotide CO phosphate dehydrogenase NADH homocysteine 2 (UMP synthase) (multiple NADPH (DHFR) NH + (AIR) (in adrenal cortex, some in gonad) (in peripheral tissue) ATP ADP glyceraldehyde 4 adenosine nucleotide salvage steps) NADH Cystathionine serine NADP+ Glycine B AIR carboxylase CO2 1,3-bisphosphoglycerate CO β-synthase 6 Fatty acid synthesis 2 cleavage uridine-5'-monophosphate serine CoA lanosterol ADP complex (UMP) protein glycerol-3-phosphate (multiple steps) Phosphoglycerate sphingosine sphingolipids NAD+ cystathionine carboxyaminoimidazole ribonucleotide modication protein kinase 10 O ATP THF (CAIR) 2 modication Serine hydroxymethyltransferase glutathione ATP CO2 Bisphosphoglycerate Phosphoglycerate Phosphoserine Phosphoserine B (SHMT) B Cystathionine + dehydrogenase transaminase 6 phosphatase 6 UMP kinase 4 NAD 8 NADPH Malonyl-CoA ACP transacylase mutase glycine nucleotides B ATP aspartate (multiple malonyl-ACP malonyl-CoA 3-phosphoglycerate 3-phopsphopyruvate 3-phosphoserine serine γ-lyase 6 (in mitochondrial matrix) fatty acyl-ACP α-ketobutyrate succinyl-CoA ADP steps) Glycerol-3-phosphate P TCA SAICAR synthetase 4 NADH 8 NADP+ palmitoyl-CoA CoA ACP i Phosphoglycerate NAD+ NADH glutamate α-ketoglutarate H O P lysine porphyrins ADP acyltransferase CoA 2 i arginine glutathione mutase Amidinotransferase cysteine H O (GPAT) ADP phosphatidylserine α-ketoglutarate 2 2 CO Pi 2 β-ketoacyl-ACP B (in mitochondrial matrix) Coenzyme A Pi UDP 7 Acetyl-CoA Malonyl-CoA Lysine-α- formate (rst cycle only) NADPH ornithine + Threonine dehydratase synthase carboxylase decarboxylase 2,3-bisphosphoglycerate 2-phosphoglycerate O2 NH ATP ceramide ketoglutarate Cysteine 4 2,3-bisphosphoglycerate succinylaminoimidazole carboxamide ribonucleotide 3 7-dehydrocholesterol CoA ACP CO reductase dioxygenase ATP 3 (7 cycles) 2 phosphatase + Nucleoside HCO - CO sphingosine NADP guanidinoacetate (SAICAR) 3 2 threonine diphosphate NADPH PP ACP β-ketoacyl-ACP Acetyl-CoA ACP Enolase saccharopine B phosphatidic acid i Long-chain Transaminase 6 ADP kinase synthase transacylase H2O + 7-Dehydro- NAD SAM cysteine β-sulnylpyruvate pyruvate + fatty β-ketoacyl-ACP acetyl-ACP acetyl-CoA Guanidinioacetate NADP GTP GDP CO Branched chain amino acid breakdown H2O cholesterol H O AMP 2 Saccharopine sulnate Adenylosuccinate lyase 2 acyl-CoA α-ketoglutarate NADH methyltransferase glutamate 2- dehydrogenase -ketoglutarate sulte (SO3 ) reductase ACP