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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 alanine Pi α synthetase NADPH CO2 CoA oxaloacetate phosphoenolpyruvate (GAMT) SAH ADP fumarate β-ketoacyl-ACP glutamate ATP UTP Pi phospholipids ATP Phosphoenolpyruvate glutamate phosphocreatine cholesterol reductase Cysteine sul nite + carboxykinase Pyruvate ADP (spontaneous) Sul te Triacylglycerol NADP B leucine isoleucine valine α-aminoadipic semialdehyde decarboxylase CO ATP NADH Alanine 6 Creatine 2 aminoiminidazole carboxamide ribonucleotide 1,2-diacylglycerol Malate kinase NAD+ oxidase CTP synthetase synthetase β-hydroxyacyl-ACP Fatty acid synthase P transaminase (ALT) kinase creatinine Hypotaurine (AICAR) CoA i glutamate dehydrogenase ATP α-ketoglutarate α-ketoglutarate α-ketoglutarate Dehydrogenase creatine complex + + dehydrogenase NAD (excreted) 2- ADP + Pi + ADP NADP NADPH CO2 H2O hypotaurine taurine bile salts sulfate (SO ) NH fatty acyl-CoA Aspartate Transaminase Transaminase Transaminase NADH 4 4 Lactate dehydrogenase 10 palmitate (C16) β-hydroxyacyl-ACP Citrate lyase amino- B B B N -formyl-THF malate pyruvate lactate 6 6 6 α-aminoadipate NAD+ NADH glutamine vitamin D bile salts cell lipoproteins dehydratase H O transferase glutamate glutamate glutamate AICAR transformylase (spontaneous) CoA 2 ATP CoA Malic enzyme (spontaneous) (skin) (liver) membranes B (AST) + α-ketoglutarate 6 NADH NAD THF B glutamate trans-enoyl-ACP α-ketoglutarate Transaminase 6 triacylglycerol citrate aspartate α-ketoisocaproate α-keto-β-methylvalerate α-ketoisovalerate glutamate Urea cycle Enoyl-ACP NADPH oxaloacetate formaminoimidazole carboxamide ribonucleotide CTP reductase α-ketoadipate (FAICAR) NH + HCO - NADP+ tricarboxylate glutamate/ dicarboxylate mitochondrial pyruvate 4 3 glutamate transporter aspartate transporter transporter carrier Transaminase (n+2) fatty acyl-ACP Fatty acid oxidation Argininosuccinate IMP cyclohydrolase Citric acid cycle (tricarboxylic acid cycle) citrulline B H O α-ketoisocaproate α-keto-β-methylvalerate α-ketoisovalerate -ketoadipate 6 2 lipoproteins α synthetase α-ketoglutarate - 2 ATP ornithine/citrulline HCO PPi (extrahepatic metabolism) free fatty acids free fatty acids aspartate α-ketoglutarate 3 NAD+ CoA Branched-chain NAD+ CoA NAD+ CoA NAD+ CoA Carbamoyl transporter CoA ATP AMP PP pyruvate ATP i citrate ketoacid B B B B aspartate 1 BCKAD 1 BCKAD 1 α-ketoadipate 1 phosphate 2 ADP inosine monophosphate B AMP Diet fatty acids fatty acids Pyruvate 7 NADH CO dehydrogenase NADH CO NADH NO fatty acids fatty acyl- CoA CoA 2 2 CO2 dehydrogenase synthetase I (IMP) > C14 and < C22 Long-chain fatty carboxylase (BCKAD) + NH + > C24 to peroxisomes ≤ C12 CO2 NADH CO (CPS1) citrulline Nitric oxide glutamine NH 4 AST 2 Pi 4 Legend acyl-CoA synthetase ATP NAD+ synthase Asparagine synthetase AMP deaminase Acyl-CoA B B Adenylosuccinate H2O carnitine 6 Pyruvate 1 isovaleryl-CoA α-methylbutyryl-CoA isobutyryl-CoA glutaryl-CoA Ornithine P GTP 4 lysine Synthetase CPT1 ADP i glutamate synthetase dehydrogenase transcarbamoylase (OTC) NADP+ NAD+ Carbohydrates NADH NAD+ GDP Amino acids P carbamoyl Pi IMP dehydrogenase i Isovaleryl-CoA H2O (multiple steps) FAD argininosuccinate + CoA Glutaryl-CoA phosphate NH ATP + carnitine transporter carnitine fatty acyl-carnitine dehydrogenase 4 NADH NADP glutamate oxaloacetate NADH acetyl-CoA NADPH aspartate ethanol dehydrogenase polyamines ornithine Asparaginase GMP reductase Oxidative (spontaneous) FADH2 O2 Nucleotides + Translocase H2O AMP NADPH metabolism Alcohol NAD CPT2 β-methylcrotonyl-CoA propionyl-CoA Argininosuccinate Base salvage dehydrogenase odd-chain fatty crotonyl-CoA oxaloacetate CoA ATP AMP PP acetyl-CoA (last three carbons only) ornithine/citrulline lyase PP NADH i - - acyl-CoA i Heme HCO HCO B Lipids 4 3 3 Ornithine aminotransferase 6 transporter adenylosuccinate XMP metabolism fatty acids ≤ C12 fatty acyl-CoA CoA ATP ATP H O acetaldehyde β-methylcrotonyl-CoA Propionyl-CoA 2 Hydratase aspartate arginine fumarate asparagine Fatty acyl-CoA synthetase ornithine Arginase PP Hypoxanthine-guanine carboxylase B carboxylase B i (long chains only) 7 7 ATP ADP P glutamine Multiple steps FAD ADP ADP i Adenylosuccinate phosphoribosyltransferase ATP black dashed arrow Acyl-CoA citrate β-hydroxybutyryl CoA TCA Citrate H O CoA glutamate glutamic semialdehyde urea H2O (HGPRT) GMP synthetase Ketone body metabolism dehydrogenases 2 lyase PRPP H O β-methylglutaconyl-CoA methylmalonyl-CoA AMP + PP Participates in additional reactions FADH oxaloacetate synthase 2 + glutamate i gray dashed arrow acetaldehyde 2 NAD NADPH NADP+ NADH Aconitase fumarate + β-methylglutaconyl-CoA Methylmalonyl-CoA Dehydrogenase (spontaneous) NAD trans-enoyl-CoA Pyrroline-5- + hypoxanthine Acetaldehyde + NADPH NADP Multiple cycles NAD cis-aconitate hydratase mutase B12 heavy gray arrow dehydrogenase acetyl-CoA x2 NADH carboxylate synthetase Malate H O proline Adenine NADH H O 2 pyrroline- pyrroline-5-carboxylate Crosses membrane 2 succinyl-CoA dehydrogenase º-hydroxy-β-methylglutaryl-CoA acetoacetyl-CoA Pyrroline-5-carboxylate phosphoribosyltransferase HGPRT thin gray arrow Hydratase Aconitase succinyl-CoA 5-carboxylate AMP adenine guanine GMP acetate (HMG-CoA) reductase Thiolase (many cycles) CoA Outer mitochondrial membrane CoA CoA Thiolase PP PP isocitrate HMG-CoA lyase i PRPP PRPP i β-hydroxyacyl-CoA Methylmalonyl-CoA mutase B12 ATP Inner mitochondrial membrane Acetyl-CoA malate Adenylate ATP ATP acetoacetyl-CoA + + Nucleotide breakdown Deoxyribonucleotides 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 PP dehydrogenases dehydrogenase Nucleases ATP GTP CTP UTP ATP i acetyl-CoA NADH NADH ADP Ammonium capture and release B6 HMG-CoA synthase (in liver) CO2 Glutaminase B ribose-5-P Propionyl-CoA carboxylase 7 + H2O H2O H2O H2O acetyl-CoA + Vitamins CoA β-ketoacyl-CoA NH4 H2O ADP GDP 5 H O Fumarase NH4 H2O 5 ATP 2 (deoxy) GMP (deoxy) AMP (deoxy) CMP UMP deoxyTMP Phosphatase Phosphatase Phosphatase Phosphatase Enzyme cofactor: P P P P CoA glutamate glutamine i i i i Phosphopentomutase Thiolase - α-ketoglutarate H O H O H2O H2O α-ketoacid dehydrogenases and HCO 2 H2O 2 uracil Pi B thiamine -hydroxy- -methyl-glutaryl-CoA 3 1 transketolase β β + Nucleotidase Nucleotidase Nucleotidase Nucleotidase Nucleotidase Nucleoside GTP ATP Nucleoside (odd-chain fatty acids only) NADH NAD NH + H O ATP ADP P (HMG-CoA) 4 2 i P P P ADP GDP CDP UDP ribose-1-P uridine Precursor: Glutamate P P H O i i i diphosphate diphosphate propionyl-CoA Glutamine synthetase i i 2 Uridine phosphorylase B riboavin α-ketoglutarate CoA dehydrogenase-1 AMP kinase GDP ADP kinase 2 FAD/FADH2 (n-2) fatty acyl-CoA acetyl-CoA fumarate HMG-CoA lyase (in liver) B NAD+ deaminase TCA dehydrogenase 1 N-acetylglutamate synthase NH + NH + Precursor: (deoxy) guanosine 4 4 B niacin + + cholesterol (cytosol) many many 3 NAD /NADH and NADP /NADPH CO2 acetyl-CoA CoA CO2 amino Transaminases B6 α-ketoacids (deoxy) IMP (deoxy) (deoxy) thymidine NADH Purine P Pyrimidine ATP S-adenosyl methionine GTP B pantothenate Precursor: CoA acids i cytidine uridine dADP dGDP dCDP dUDP biopterin 5 acetyl-CoA acetoacetate acetone Glutamate N-acetylglutamate nucleoside H O nucleoside (spontaneous) 2 P decarboxylase CO phosphorylase (deoxy) Nucleotidase deaminase Thymidine i Coenzyme A Enzyme cofactor: heme b 2 ATP ATP ATP ATP pyridoxal acetate H O ribose-1-P P phosphorylase B transaminases and other reactions succinyl-CoA 2 Adenosine i deoxy 6 phosphate NADH TCA 2 H+ glutamate α-ketoglutarate Hydrolase urea cycle (regulation) Kinase Kinase Kinase Kinase involving amino acids ribose-1-P NADH, FADH2 β-hydroxybutyrate deaminase Uridine Pi Heme synthesis Pi guanine ADP ADP ADP ADP dehydrogenase Ferrochelatase Succinyl-CoA (deoxy) (deoxy) + 2+ succinic semialdehyde γ-aminobutyric + phosphorylase biotin Enzyme cofactor: carboxylases NAD Fe synthetase 3 H adenosine inosine (deoxy) B GDP + GABA acid (GABA) H O NH + 7 glycine succinyl-CoA NAD 2 4 ribose-1-P protoporphyrin IX Succinic semialdehyde aminotransferase ADP + P ATP (spontaneous) GTP i Nucleoside salvage + dehydrogenase B dATP dGTP dCTP dUTP NAD NADH succinyl-CoA succinate CoA 6 ATP ADP NADH Pi uracil thymine folate Single-carbon carrier + Purine nucleoside + B CoA 3 H2O2 δ-aminolevulinic Protoporphyrinogen 4 H NH4 9 β-hydroxybutyrate acetoacetate acetoacetyl-CoA acetyl-CoA x2 + CoA phosphorylase (deoxy) adenosine adenosine 5’-monophosphate (AMP) B NADH NAD + Oxidative phosphorylation V NADPH NADPH CO acid synthase 6 + Adenosine kinase 2 Succinyl-CoA:acetoacetate Thiolase IX oxidase succinate 4 H 2 H ribose-1-P Dihydropyrimidine H O -hydroxybutyrate 3 O Pi ADP ATP 2 Enzyme cofactor: β 2 dUTP transferase (SCOT) 1 + + dehydrogenase + Phosphatase cobalamin methylmalonyl-CoA mutase and dehydrogenase /2 O2 + 2H H2O NADP NADP pyrophosphatase B12 hypoxanthine P PP ATP ADP homocysteine methyltransferase δ-aminolevulinic acid (δ-ALA) (in extrahepatic tissues) protoporphyrinogen IX I (DPD) i i FADH FAD 2 II Thymidylate cytidine cytidine 5’-monophosphate (CMP) H O + Uridine-cytidine kinase ------III - - H O 2 NH synthase ATP ADP 2 e 2 e 2 e 2 Guanine dihydrouracil dihydrothymine 4 I IV phosphate adenine nucleotide Xanthine (TS) uridine uridine 5’-monophosphate (UMP) CoQ transporter O Cyt c transporter deaminase 2 dCMP dUMP dTMP dTDP 2 e- + oxidase NH4 Dihydropryimidinase Deoxynucleotide Kinase ATP ADP 6 ++++++++++++++++++++++++++++++++++++++++++++++++++++++++ 6 protoporphyrinogen IX Cytochrome c deaminase NADH Succinate Cytochrome c ATP synthase H2O2 ATP ADP 2 CO 2 H O + -ureidopropionate -ureidoisobutyrate thioredoxin 5 10 2 2 4 H dehydrogenase dehydrogenase reductase oxidase (Complex V) xanthine β β N ,N - P ADP ATP (reduced) ATP (Complex I) (Complex II) (Complex III) + (Complex IV) + + i dihydrofolate deoxycytidine deoxycytidine 5’-monophosphate (dCMP) 4 H 2 H 3 H methylene-THF Kinase Pathways of O Deoxycytidine kinase 2 + Coproporphyrinogen oxidase O2 H2O SHMT ADP CO2 CO2 NADP DHFR 2 H2O H2O H2O 4 CO2 Ureidopropionase Thioredoxin Heme degradation + + ATP ADP Human Metabolism 3 NADPH 3 NADP NADPH NADP UDP-glucuronate x2 UDP x2 feces Xanthine oxidase reductase NADPH glycine NADPH δ-ALA x2 porphobilinogen (PBG) x4 hydroxymethylbilane uroporphyrinogen III coproporphyrinogen III H O NH + NH + Ribonucleotide VERSION 10.18 heme b biliverdin bilirubin conjugated bilirubin 2 2 4 4 reductase THF thymidine deoxythymidine-5'-monophosphate (dTMP) δ-ALA dehydratase PBG deaminase Uroporphyrinogen III Uroporphyrinogen urine + Thymidine kinase + thioredoxin NADP 4 NH4 synthase decarboxylase Biliverdin reductase UDP-glucuronyltransferase serine deoxyuridine deoxyuridine 5’-monophosphate (dUMP) Email questions or comments to: [email protected] 3 O Fe 3+ CO bile β-alanine β-aminoisobutyric (oxidized) dTTP 2 uric acid 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