A B Cholesterol synthesis Glycogen and galactose metabolism acetyl-CoA x2 1 2 3 4 5 6 8 9 CoA acetoacetyl-CoA acetyl-CoA HMG-CoA synthase CoA glycogen (n) 6 3 4 5 α-1,6 bond Steroid hormone synthesis CoA UDP-glucuronate Cholesterol desmolase 17α-hydroxylase pregnenolone CO2 isopentenyl pyrophosphate (IPP) (C5) x3 (multiple steps) galactitol Aromatase 11β-hydroxysteroid dehydrogenase 2 (11β-HSD2) + (multiple steps) Glycerol-3-phosphate acyltransferase (GPAT) NADPH NADP+ glycerol Triacylglycerol synthetase complex protein modification 2 3 4 2' 3' 4' ATP glutamine β-ketoacyl-ACP synthase ATP Phosphofructokinase-1 Glutamine: F6P aminotransferase B6 ADP phosphatidic acid PPi H2O AMP ATP phospholipids 1,2-diacylglycerol Long-chain fatty acyl-CoA synthetase β-ketoacyl-ACP CoA fatty acyl-CoA palmitate (C16) β-hydroxyacyl-ACP dehydratase ADP carboxylase dihydroxyacetone phosphate (DHAP) fatty acids ≤ C12 Triosephosphate isomerase fatty acids > C24 to peroxisomes Nucleotides HCO3 Heme metabolism 4 dehydrogenase Aspartate aminotransferase B6 (AST) citrate aspartate tricarboxylate transporter Fatty acid oxidation NAD + NADP+ NADPH malate PPi citrate fatty acyl- CoA Long-chain fatty acyl-CoA synthetase Multiple cycles Crosses membrane Fatty acyl-CoA synthetase Acyl-CoA dehydrogenases Hydratase CoA Acetyl-CoA synthetase 5 PPi Hydroxyacyl-CoA dehydrogenases HMG-CoA synthase (in liver) CoA Thiolase Enzyme cofactor: transaminases and other reactions involving amino acids Heme synthesis Enzyme cofactor: carboxylases glycine succinyl-CoA acetoacetate NADH NAD+ CoA CO2 Enzyme cofactor: methylmalonyl-CoA mutase and homocysteine methyltransferase δ-aminolevulinic acid synthase B6 δ-aminolevulinic acid (δ-ALA) VERSION 10.23 Copyright 2017 Stanford School of Medicine. All rights reserved. This work is licensed under a Creative Commons AttributionNonCommercial-NoDerivatives 4.0 International License. lactate (spontaneous) B6 B6 B6 glutamate α-keto-β-methylvalerate α-ketoisovalerate CO2 NAD+ Pyruvate B1 dehydrogenase NADH NAD NADH NAD+ CoA NADH Citrate synthase oxaloacetate CoA H2O H2O Aconitase Propionyl-CoA carboxylase B7 CoA NAD Isocitrate dehydrogenase ADP H2O HCO3 NH4 Fumarase CoA α-ketoglutarate B dehydrogenase 1 many amino Transaminases B6 acids NADH succinyl-CoA Pi Succinyl-CoA synthetase GDP Fe2+ protoporphyrin IX CoA Thiolase acetyl-CoA x2 3 H2O2 3 O2 Protoporphyrinogen IX oxidase NADH H2O H2O 4 CO2 hydroxymethylbilane uroporphyrinogen III Uroporphyrinogen III Uroporphyrinogen 4 NH4+ synthase decarboxylase C D succinic semialdehyde Succinic semialdehyde dehydrogenase NADH NAD CoA + 2 H2O Coproporphyrinogen oxidase + - 4H + + I - + - + - + - - + - + FADH2 II - - - - - - + + 2 e- FAD - - - - - - - - - -- 2e CoQ + NADH dehydrogenase (Complex I) + + + + + + + + - + + - - -III - - CO2 - - + + + + + + + + + γ-aminobutyric acid (GABA) -- - - - - - - - 2e - + + + + + + + + Nucleotidase Pi H2O NADPH NADP+ biliverdin - - IV + + - + - + - + - + - - + - + ATP synthase (Complex V) - + 3 H+ - + - + - - phosphate transporter + + Pi - - - - - H2O adenine nucleotide transporter + F conjugated bilirubin UDP-glucuronyltransferase G glutamate CO2 sulfite (SO3 ) + + ADP + NH4+ + AMP deaminase (deoxy) adenosine Pi Adenosine deaminase H 2O (deoxy) ribose-1-P taurine bile salts Guanine deaminase NH4 NADH B6 ATP CTP synthetase ADP + Pi urea ATP NH4+ Asparaginase H2O Argininosuccinate lyase NADPH Arginase IMP cyclohydrolase NH4+ AMP deaminase arginine NH4+ H2O H2O UMP Nucleotidase PPi PRPP Pyrroline-5-carboxylate reductase AMP Thymidine phosphorylase NADPH NADP+ Pi dADP Pi Dihydropyrimidine dehydrogenase (DPD) H2O CO2 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 dCDP ATP Kinase ADP ribose-1-P uracil Pi Uridine phosphorylase GDP dATP β-alanine dCTP dGTP S-adenosyl methionine ATP uridine ADP 5 Nucleoside diphosphate kinase (spontaneous) Phosphatase NADP+ H 2O Pi dUTP pyrophosphatase NADH, FADH2 dUTP ATP ADP adenosine thioredoxin (reduced) β-ureidoisobutyrate H2O Thioredoxin reductase NH4+ Thymidylate synthase (TS) dUMP NADPH dTMP uridine dTDP Kinase dihydrofolate ATP DHFR ADP glycine Uridine-cytidine kinase cytidine-5’-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 10 SHMT Ribonucleotide reductase adenosine-5’-monophosphate (AMP) ATP ADP N ,N methylene-THF 5 NADP+ thioredoxin (oxidized) β-aminoisobutyric acid Deoxynucleotide deaminase Adenosine kinase Nucleoside salvage ATP ADP PPi NH4+ dCMP GTP biopterin Coenzyme A ATP Kinase ADP NH4+ NADPH CO2 ATP dUDP ATP Kinase ADP dihydrothymine Ureidopropionase NH4+ I H 2O Phosphatase Pi CDP dGDP ATP Kinase ADP thymine β-ureidopropionate H2O2 UTP H2O Phosphatase Pi GDP Dihydropryimidinase H2O CTP (deoxy) ribose-1-P dihydrouracil H2O2 Adenylate kinase Hypoxanthine-guanine phosphoribosyltransferase (HGPRT) hypoxanthine Adenine phosphoribosyltransferase proline NADP+ GMP reductase NADPH XMP NADP+ H2O Phosphatase Pi ADP thymidine uracil O2 H2O Phosphatase Pi deoxyTMP H2O H2O Nucleotidase Pi Pi (deoxy) uridine Pi H 2O NAD+ IMP dehydrogenase NADH 4 + Uridine phosphorylase (deoxy) ribose-1-P H2O fumarate GTP NH4+ adenylosuccinate H 2O pyrroline-5-carboxylate H 2O Adenylosuccinate GTP synthetase GDP Pi Adenylosuccinate lyase pyrroline5-carboxylate CTP Base salvage PPi NADPH glutamate aspartate asparagine fumarate (spontaneous) inosine monophosphate (IMP) AMP 3 NH4+ glutamine formaminoimidazole carboxamide ribonucleotide (FAICAR) glutamine Asparagine synthetase glutamate Nucleoside diphosphate kinase UTP N10-formyl-THF α-ketoglutarate argininosuccinate O2 ornithine (deoxy) cytidine Pyrimidine nucleoside deaminase uric acid H ATP Adenylosuccinate lyase AICAR transformylase aspartate (spontaneous) NH4 + (deoxy) inosine Xanthine oxidase urine bile UDP sulfate (SO42-) glutamate Transaminase Argininosuccinate synthetase PPi ATP ATP H2O hypoxanthine Xanthine oxidase feces ADP aminoiminidazole carboxamide ribonucleotide (AICAR) oxaloacetate ornithine/citrulline transporter Pi Pi Purine nucleoside phosphorylase xanthine ATP UMP kinase fumarate Sulfite oxidase Hypotaurine dehydrogenase hypotaurine ATP SAICAR synthetase H2O 2- Deoxyribonucleotides Nucleotidase Pi guanine UDP x2 bilirubin Biliverdin reductase 3 O2 Fe 3+ CO Heme oxygenase ornithine (deoxy) CMP Nucleotidase ATP ADP Pi uridine-5'-monophosphate (UMP) ADP pyruvate β-sulfinylpyruvate NADP+ H 2O Pi (deoxy) IMP H2O + Cytochrome c oxidase (Complex IV) 2 H+ UDP-glucuronate x2 Pi Ornithine transcarbamoylase (OTC) glutamic semialdehyde H 2O Pi Purine nucleoside phosphorylase CO2 Pi Transaminase B6 OMP decarboxylase (UMP synthase) CO2 succinylaminoimidazole carboxamide ribonucleotide (SAICAR) threonine Nitric oxide synthase RNA/DNA Nucleotidase (deoxy) guanosine orotidine-5'-monophosphate (OMP) aspartate ADP B6 H2O AMP citrulline Pi (deoxy) AMP O2 3 NADPH 3 NADP+ ornithine/citrulline transporter NADPH NADP+ Pyrroline-5carboxylate synthetase Dehydrogenase urea cycle (regulation) V TCA Threonine dehydratase NAD+ citrulline glutamate Nucleotide breakdown ATP ADP + Pi 2 H+ /2 O2 + 2H - creatinine (excreted) succinyl-CoA PPi 3 H+ B6 2e + acetate H2O Hydrolase O2 Cysteine sulfinite decarboxylase (spontaneous) ATP ATP - ATP ADP 2 Orotate phosphoribosyltransferase PPi aminoimidazole ribonucleotide (AIR) nucleotide salvage α-ketoglutarate H2O Ornithine aminotransferase B6 aspartate N-acetylglutamate α-ketoglutarate GABA aminotransferase Cytochrome c reductase (Complex III) 4 H+ heme b E - ADP Glutamate B6 Cyt c Succinate dehydrogenase (Complex II) Heme degradation coproporphyrinogen III - Creatine kinase polyamines N-acetylglutamate synthase acetyl-CoA CoA many α-ketoacids Oxidative phosphorylation 4 H+ succinate I phosphocreatine oxaloacetate (deoxy) GMP NH4+ H2O ATP Glutamine synthetase 1 protoporphyrinogen IX O2 glutamate NAD+ GTP 4 H+ Pi ADP ATP H2O cysteine sulfinate NO carbamoyl phosphate Dihydroorotate dehydrogenase (in mitochondria) Pi NH4+ cysteine SAM 2 ADP Hydratase glutamine NADH NAD Glutamate dehydrogenase-1 decarboxylase 2 H+ 2 CO2 PBG deaminase H2O NADH ADP (in mitochondrial matrix) α-ketobutyrate Cysteine dioxygenase B6 glutamate NAD+ CO2 Ferrochelatase succinate Glutaminase NH4+ H2O AIR synthetase Thiolase acetyl-CoA x2 TCA + fumarate ornithine Nucleases α-ketoglutarate - propionyl-CoA glutathione Coenzyme A acetoacetyl-CoA CoA ADP carboxyaminoimidazole ribonucleotide (CAIR) Cystathionine γ-lyase B6 porphyrins 2 ATP NADH succinyl-CoA NAD+ formiminoglycinamidine ribonucleotide (FGAM) cystathionine arginine crotonyl-CoA NAD+ Methylmalonyl-CoA mutase B12 Ammonium capture and release + HCO3 Carbamoyl phosphate synthetase I (CPS1) β-hydroxybutyryl CoA methylmalonyl-CoA acetyl-CoA acetoacetate NADH CO2 ATP Propionyl-CoA carboxylase B7 ADP NH4 Glutaryl-CoA dehydrogenase H2O HMG-CoA lyase + odd-chain fatty acyl-CoA (last three carbons only) HCO3- ATP β-hydroxy-β-methylglutaryl-CoA (HMG-CoA) malate NADH FAD ATP orotate AIR carboxylase Urea cycle CoA α-ketoadipate B1 dehydrogenase CO2 NADH dihydroorotate THF glutaryl-CoA isobutyryl-CoA propionyl-CoA β-methylcrotonyl-CoA carboxylase B7 isocitrate Methylmalonyl-CoA mutase B12 NADH NAD+ CH3 (used for methylation) adenosine serine (spontaneous) + Dihydroorotase PRPP S-adenosylhomocysteine (SAH) Cystathionine β-synthase B6 nucleotides SAH N-carbamoyl aspartate Pi glutamine homocysteine Transaminase B6 FADH2 β-methylglutaconyl-CoA hydratase Aconitase CoA BCKAD B1 CO2 acetyl-CoA β-methylglutaconyl-CoA H2O methylmalonyl-CoA Methylene-THF reductase creatine α-ketoadipate (multiple steps) HCO3- ADP citrate NAD+ α-methylbutyryl-CoA β-methylcrotonyl-CoA Malate dehydrogenase succinyl-CoA TCA succinyl-CoA NADH Isovaleryl-CoA dehydrogenase cis-aconitate NAD + FGAM synthetase 3 Pi N5-methyl-THF NADH α-aminoadipate glutamate α-ketoisovalerate CoA BCKAD B1 CO2 NAD+ isovaleryl-CoA heme b NADH α-keto-β-methylvalerate CoA Branched-chain ketoacid B1 CO2 dehydrogenase (BCKAD) NAD+ acetone acetoacetate acetoacetyl-CoA β-hydroxybutyrate Succinyl-CoA:acetoacetate β-hydroxybutyrate transferase (SCOT) dehydrogenase (in extrahepatic tissues) δ-ALA x2 porphobilinogen (PBG) x4 δ-ALA dehydratase Transaminase N10-formyl-THF formyl-GAR (FGAR) S-adenosylhomocysteine hydrolase glutathione Guanidinioacetate methyltransferase (GAMT) Pi THF Homocysteine B12 methyltransferase guanidinoacetate α-aminoadipic semialdehyde NAD+ Dehydrogenase Transaminase α-ketoisocaproate SAH S-adenosylmethionine (SAM) NAD+ Amidinotransferase (in mitochondrial matrix) Aspartate transcarbamoylase aspartate glycinamide ribonucleotide (GAR) 5-hydroxyindoleacetic acid NADP+ α-ketoglutarate Citric acid cycle (tricarboxylic acid cycle) SAM carbamoyl phosphate ADP TCA glycine NADP saccharopine NAD+ Saccharopine NADH dehydrogenase glutamate α-ketoglutarate glutamate CoA 1 ATP Pi melatonin Pi 2 ADP 5-phosphoribosylamine H2O NADH Glycine cleavage complex THF Lysine-αketoglutarate reductase + NAD+ + β-hydroxybutyrate dehydrogenase 2 H2O NH4+ NADPH Transaminase glutamate Hydroxyindole O-methyltransferase Carbamoyl phosphate synthetase II (CPSII) glutamate glutamate N5,N10-methylene-THF Methenyl-THF reductase α-ketoglutarate valine α-ketoglutarate α-ketoglutarate pyruvate oxaloacetate protoporphyrinogen IX B Lactate dehydrogenase NADH ADP - Email questions or comments to: metabolicpathways@stanford.edu ATP VMA GAR transformylase methionine NADPH lysine isoleucine leucine Alanine B6 transaminase (ALT) pyruvate ATP FADH2 6 Pathways of Human Metabolism ADP cholesterol (cytosol) NAD serine glutamate NADH TCA H2O acetyl-CoA pyrimidine synthesis Serine hydroxymethyltransferase (SHMT) B6 alanine (long chains only) FAD BH2 Dihydrobiopterin reductase N-acetyl-5-HT Methyltransferases N5,N10-methenyl-THF Methenyl-THF cyclohydrolase Branched chain amino acid breakdown ATP (n-2) fatty acyl-CoA acetyl-CoA + Single-carbon carrier glutamate (odd-chain fatty acids only) HMG-CoA lyase (in liver) acetyl-CoA N10-formyl-THF sphingosine α-ketoglutarate vanillylmandelic acid (VMA) 2 ATP H 2O fumarate NADPH NADP+ Pi H 2O α-ketoglutarate glutamate Fumarylacetaoacetate hydrolase H2O acetoacetate H 2O ceramide CoA CO2 Precursor: CoA glutamate NADH 3-phosphoserine PPi SAH Monoamine oxidase (MAO) fumarylacetoacetate histidine catabolism ADP Pi C1-THF synthase Phosphoserine phosphatase Translocase β-ketoacyl-CoA β-hydroxy-β-methyl-glutaryl-CoA (HMG-CoA) Precursor: NAD+/NADH and NADP+/NADPH fatty acyl-carnitine β-hydroxyacyl-CoA AMP TCA tetrahydrofolate (THF) DHFR Phosphoserine transaminase B6 3-phosphopyruvate (many cycles) acetoacetyl-CoA acetyl-CoA Enzyme cofactor: α-ketoacid dehydrogenases and transketolase Precursor: FAD/FADH2 Thiolase ATP acetyl-CoA ATP formate glutamine Methionine adenosyltransferase phosphatidylserine Pyruvate B7 carboxylase B6 CPT1 fatty acyl-CoA NADH CoA Phosphoglycerate dehydrogenase NAD+ CO2 HCO3- α-ketoglutarate acetyl-CoA x2 acetate ATP mitochondrial pyruvate carrier dicarboxylate transporter trans-enoyl-CoA NAD+ (spontaneous) CO2 acetyl-CoA acetaldehyde Acetaldehyde dehydrogenase GTP BH4 SAM Serotonin N-acetyltransferase CO2 NADPH NADP epinephrine H2O serotonin (5-HT) niacin Maleylacetoacetate isomerase (spontaneous) ADP Pyruvate kinase PPi Ketone body metabolism Participates in additional reactions Vitamins ATP AMP CoA Multiple steps succinylacetoacetate TCA α-ketoisocaproate AST Acyl-CoA Synthetase 5-hydroxytryptophan HCO3glutamine GAR synthetase Aromatic amino acid decarboxylase B6 (DOPA decarboxylase) xylulose-5-phosphate (step 1) dihydrofolate Dihydrofolate reductase (DHFR) NADH phosphoenolpyruvate Malic enzyme CoA CPT2 fatty acids ≤ C12 H2O CO2 NADPH NADP+ α-ketoglutarate glutamate/ aspartate transporter aspartate Homogentisate dioxygenase maleylacetoacetate α-ketoadipate (spontaneous) acetaldehyde B9 folate 3-phosphoglycerate Phosphoenolpyruvate carboxykinase NADH Malate dehydrogenase glutamate CoA tryptophan THF NAD+ Glyceraldehyde-3phosphate dehydrogenase oxaloacetate ADP Citrate lyase ATP ATP AMP O2 O2 homovanillic acid (HVA) H2O Pyrimidine synthesis Glutamine PRPP amidotransferase (GPAT) glycine Tryptophan hydroxylase Single-carbon metabolism and sulfur-containing amino acids 2-phosphoglycerate 2,3-bisphosphoglycerate 2,3-bisphosphoglycerate phosphatase Enolase H2O CO2 GTP GDP CO2 - Pi carnitine NADH (step 2) O2 Methyltransferase norepinephrine CO2 BH2 Dihydrobiopterin reductase Dopamine β-hydroxylase dopamine ATP Phosphoglycerate mutase Pi acetyl-CoA Fatty acid synthase lysine ethanol NAD+ Alcohol Ribulose-5phosphate epimerase BH4 + GTP-cyclohydrolase 7 5 + + Transketolase B1 3 5 glyceraldehyde3-phosphate Tyrosine hydroxylase NADP+ NADPH homogentisate (spontaneous) formate ribose-5-phosphate NADPH NADP+ Pi Triose kinase ADP ATP Malonyl-CoA decarboxylase ATP NADPH CoA fatty acids > C14 and < C22 Ribose-5phosphate isomerase glyceraldehyde3-phosphate Bisphosphoglycerate mutase trans-enoyl-ACP Enoyl-ACP reductase alkapton Dihydroneopterin triphosphate purine synthesis CoA ACP H2O CO2 CO2 ribulose-5-phosphate B6 p-hydroxyphenylpyruvate dioxygenase M 5-phosphoribosyl pyrophosphate (PRPP) Aromatic amino acid decarboxylase B6 (DOPA decarboxylase) Aldolase NADP+ β-hydroxyacyl-ACP erythrose4-phosphate 4 + 5 3 FADH2 malonyl-CoA Pi Acetyl-CoA:ACP transacylase O2 1,3-bisphosphoglycerate B7 Acetyl-CoA CO2 NADPH β-ketoacyl-ACP reductase ATP H2O dihydroxyphenylanine (L-DOPA) p-hydroxyphenylpyruvate (multiple steps) (step 3) glyceraldehyde ACP acetyl-ACP AMP 5-phosphoribosyl pyrophosphate (PRPP) Dihydrobiopterin reductase NADP+ NADPH 6-Phosphogluconate dehydrogenase Dihydrobiopterin (BH2) succinylacetone fructose-1,6-bisphosphate Fructose-1-P aldolase (aldolase B) fructose-1-phosphate Malonyl-CoA:ACP transacylase β-ketoacyl-ACP synthase Lactonase O2 Transaminase glutamate L Purine synthesis α-ketoglutarate Tetrahydrobiopterin (BH4) 6-phosphogluconate (multiple steps) tyrosine α-ketoglutarate H 6 7 + + Transaldolase 4 3 6 ADP CO2 ACP glutamate phenylalanine + sedoheptulose7-phosphate Transketolase B1 + Coenzyme Q (oxidized) NAD NADH Fructokinase (first cycle only) ACP (7 cycles) phenylpyruvate K melanin Tyrosinase (multiple steps) H2 O Phenylalanine hydroxylase J dopaquinone triiodothyronine (T3) thyroxine (T4) NADP+ NADPH PRPP synthetase Pi Fructose-1,6bisphophatase O2 Transaminase B6 NADP+ NADPH H2O 6-phosphogluconolactone nucleotides Phosphoglycerate kinase malonyl-ACP Glutathione peroxidase 2 H2O Phosphomannose isomerase fructose-6-phosphate mannose-6-phosphate Hexokinase Dehydrogenase phenyllactate biosynthetic reduction reactions Glucose-6-phosphate dehydrogenase (G6PD) Phosphoglucose isomerase sphingolipids sphingosine NADPH NADP+ Phosphofructokinase-2 Fructose bisphosphatase-2 (Bifunctional enzyme) (multiple steps) H2O2 Glutathione reductase glutathione (oxidized) ADP glucose-6-phosphate + ATP carnitine transporter Plasma membrane cobalamin 4' Pi Inner mitochondrial membrane B12 1 Glycerol-3-phosphate dehydrogenase glycerol3-phosphate CoA free fatty acids Outer mitochondrial membrane folate 3' Amino acids Lipids B9 2' Fatty acid synthesis carnitine Oxidative metabolism biotin 4 glutathione (reduced) I Aromatic amino acid metabolism phenylacetate ATP ADP fructose palmitoyl-CoA CoA free fatty acids Carbohydrates B7 0 glucosamine-6-phosphate NADPH CO2 CoA Legend B6 Glycerol kinase ATP ADP fatty acyl-ACP Pi Diet pyridoxal phosphate Hexokinase or glucokinase ADP FAD NAD NADH (multiple steps) CoA lipoproteins (extrahepatic metabolism) pantothenate 3 Glucose-6-phosphatase (in endoplasmic reticulum) ATP fructose Pentose phosphate pathway fructose-2,6-bisphosphate estradiol (n+2) fatty acyl-ACP niacin 4 (in mitochondrial matrix) NADP+ riboflavin 2 glutamate CoA triacylglycerol B2 B3 B5 1 Coenzyme Q (reduced) dihydrotestosterone (DHT) NADP+ formate cell lipoproteins bile salts (liver) membranes thiamine -1 ATP galactose (in peripheral tissue) glycerol-3-phosphate cholesterol B1 -2 mannose Galactokinase estrone + 11β-HSD1 7-dehydrocholesterol thin gray arrow ADP Aldose reductase cortisone protein modification 8 NADP heavy gray arrow 3 H NADH NAD+ α-1,6-Glucosidase Galactose-1-phosphate uridyltransferase (GALT) 17β-HSD Aromatase 2 Phosphoglucomutase GAGs androstenedione 17β-hydroxysteroid dehydrogenase (17β-HSD) serine 4 NADH gray dashed arrow 0 galactose-1-phosphate dehydroepiandrosterone (DHEA) 3β-hydroxysteroid dehydrogenase 1 + 8 NADPH black dashed arrow -1 NADP+ Glycolysis and Gluconeogenesis Lactose synthase (inside Golgi of mammary cells) glycosaminoglycans (GAGs) glycoproteins NADP+ NADPH glycolipids (in adrenal cortex, some in gonad) 4 NAD+ vitamin D (skin) + 5α-reductase aldosterone 10 O2 7-Dehydrocholesterol reductase 0 Mitochondrial glycerol3-phosphate dehydrogenase (flavoprotein) Aldosterone synthase lanosterol 2 CO2 -3 UDP-galactose glucose -2 glucose-1-phosphate UDP lactose testosterone cortisol NADPH -3 -4 -5 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 -1 1 Glucose 1' (free) UTP glucoseUTP 1-phosphate uridyltransferase UDP Zona fasciculata and zona reticularis 3 ADP 3 2 NADH 3 ATP (multiple steps) UDP-glucose UDP-glucose dehydrogenase cholesterol 2 NADP+ mevalonate 2 glycogenin -4 -5 UDP-galactose epimerase (8 cycles) -2 1' glycogen (n) primed glycogenin Zona glomerulosa 2 NADPH HMG-CoA reductase -3 4:4 Transferase PPi other glucuronidation reactions β-hydroxy-β-methylglutaryl-CoA (HMG-CoA) -4 G Sorbitol dehydrogenase sorbitol Pi 1' -5 UDP conjugated bilirubin NADPH 3' 2' UDP-glucose 7 Aldose reductase 4' Debranching enzyme phosphorylase Glycogen synthase F glucose Glycogen B6 (many cycles) 8 2 Pi 10 9 E (branch point only) (debranching) 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
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