Inborn Errors of Metabolism - Sigma-Aldrich -CO-OCH 1.1.1.30 CH 33COCH CH32COCH COO CH 32CH(OH)CH...
1
2H + H + OXALOACETATE PYRUVATE SUCCINYL-CoA GLUTARATE CITRATE MALATE 2-OXO- ASPARTATE NO 2 - NO 3 - N 2 NH 4 CH3COSCoA ACETYL-CoA HOH CO 2 NAD T R A N S A M I N A T I O N 2H + 2H + + CO 2 5-Amino- levulinate Glycine H + H + H + H + H+ H+ H+ H+ F0 F1 F1 β 2 1 α β α γ c-s u b - u n it s A D P + P i 3 .6.1.3 4 H + -tr a n s p o rtin g A T P s y n t h a s e α δ F 6 oscp ATP ADP + Pi FUMARATE UQH 2 UQ UQH 2 NADH+H + CH2COO - C(OH)COO - CH2COO - CH(OH)COO - CHCOO - 2COO - - OOCCOCH2CH2COO - -OOCCH2CH2CO.SCoA - OOCCOCH2COO- CH3CH(OH)CH2CO.SCoA - OOCCH=CHCOO - Glyoxylate Cycle -OOCCHO GTP GDP ATP + HEME Protoporphyrinogen Coproporphyrinogen Uroporphyrinogen Porphobilinogen 5-Amino- levulinate COO- CH2 CH2 H2NCH2C=O FADH 2 FAD Cyt.b Fe-S SUCCINATE II CH3COCOO- PYRUVATE P i X Y Cyt.c1 Fe-S 2e- 2e- 1e- 1e- III 2UQ UQ UQH 2 2UQH 2 2UQ. _ UQ. _ Cyt.b L Cyt.b H Cyt.c IV Cu A Cu B Cu A 2e- Heme a Heme a 3 2H + 2H + 4H + 4H + 2H + NAD + NADH+H + NAD + 1.1.1.39 1.2.4.1 2.3.1.12 3.1.3.43 4.1.1.32 2.3.3.1 4.2.1.3 1.1.1.41 1.2.4.2 2.3.1.61 6.2.1.4 -OOCCH2CH2COO- 1.3.5.1 4.2.1.2 1.1.1.37 6.4.1.1 2.3.3.8 4.1.3.1 2.3.3.9 ISOCITRATE 2.6.1.1 2.3.1.16 4.1.1.71 1.2.1.16 4.3.1.1 1.4.1.2 1.4.1.14 6.3.5.4 1.7.1.1 1.7.99.4 1.7.7.1 1.7.1.4 1.18.6.1 2.3.1.37 1.10.2.2 1.10.2.2 γ ε δ β 3 3 β A T P ADP E N D E R G O NIC REA C TIO N 1.6.5.3 I FMNH2 FMN I 1.6. 5.3 2Fe -S (5 Clusters) H + H + H + H + ATP 4H + 2H + 2H + or H+ PHOTO- SYSTEM l O 2 3.6.3.14 NADP + Glyceraldehyde Ribulose-1,5-bis-P 2 β 8 8 H + c 3 2 ε a 3 2 α 1 α ε α β 2 1 3 α α β 3 α H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H + H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ Translocated protons H+ H+ H+ H+ P i Ferredoxin 4H + PC PC PC PQH 2 2PQH 2 PQ _ . 2PQ _ . Fe-S Cyt.f PQ 2PQ Cyt bf 2e- 1e- 1e- 2e- 2e- 2e- 2e- C y clic P h oto p h o s p h o rylatio n PQ PHOTO- SYSTEM II Cyt bc N o n - c y c lic e le c tr o n fl o w 2e - 2e - Mn 2e- * Protons from Water H 2 O H+ H+ 2H + 2H + γ CO 2 Fixation H+ ADP Pi H+ NADPH+H + ATP THYLAKOID MEMBRANE CHLOROPLAST OUTER MEMBRANE THYLAKOID LUMEN STROMA (ele ctric c u rre n t) P680 Chl.a Q A Q B Pheophytin P700 Chl.A 0 A 1 Fe-S ATP ADP ATP 1 / 2 O 2 H 2 O PQH 2 To Brain - A M I N O A C I D S P Y R I M I D I N E S P U R I N E S C A T E C H O L A M I N E S A R O M A T I C A M I N O A C I D S hv VISION L I P I D P H O T O S Y N T H E S I S P O R P H Y R I N S S T E R O I D S I S O P R E N O I D S P H O S P H O L I P I D S D E G R A D A T I O N L I P I D B I O S Y N T H E S I S P E N T O S E S H E X O S E S P O L Y S A C C H A R I D E S CO CH CH(NH )COO 2 3 + FOLIC ACID C1 POOL VALINE ATP ATP 4.1.1.4 1.1.1.30 CH COCH 3 3 CH COCH COO 3 2 CH CH(OH)CH COO 3 2 3-OH-Butyrate Acetone Acetoacetate KETONE BODIES HYALURONIC ACID DERMATAN BLOOD GROUP SUBSTANCES PEPTIDO- GLYCAN N-Ac-Neuraminate (Sialate) UDP- Iduronate UDP-N-Ac- Galactosamine UDP- Galacturonate GDP-Fucose TDP-Rhamnose ADP- Glucose UDP-Glucose TDP-4-Oxo- 6-deoxyglucose MANNOSE CHITIN CHONDROITIN PECTIN ALGINATES INULIN CELLULOSE O-ANTIGENS STARCH GLYCOGEN LACTOSE GALACTOSE O OH HO OH OH CH 3 O OPPT O OH OH O OH ACNH HO OH O OH HO OPPU NHAC OH OH OH OH HO OH NHCOCH OH HO O OPPG CH OH 2 HO P UDP-N-Ac- Glucosamine pyruvate N-Ac-Mannosamine-6-P UDP-Glucuronate GDP-Mannose N-Ac-Mannosamine UDP-N-Ac-Glucosamine N-Ac-Glucosamine-6-P Sorbitol Fructose-1-P Erythrose-4-P 3-Dehydrogulonate L-Xylose L-Lyxose D-Xylulose D-Ribose Ribitol L-Arabitol L-Xylulose L-Ribulose L-Ribulose-5-P L-Xylulose-5-P L-Arabinose Xylitol D-Xylose D-Arabinose D-Ribulose ASCORBATE 2, 3-Dioxogulonate N-Ac-Glucosamine-1-P Inositol-P Inositol Glucuronate Gulonate Gulonolactone 2-Oxogulonolactone Fructose 6-P-Gluconate D-Ribulose-5-P D-Xylulose-5-P Mannose-1-P GDP-Glucose TDP-Glucose UDP-Galactose Galactose-P Glucose-1-P Glucose-6-P Fructose-6-P Fructose- 1:6-bis-P OH H H H O HOCH 2 C C C C CO OH OH H OH H H O HOCH 2 C C C CO CO OH H H OH H C C OH CO P-Ribosyl amine Glycerate 2, 3-Diphospho- glycerate 3-P-Glyceraldehyde 1:3-bis-P-Glycerate 3-P-Glycerate 2-P-Glycerate P-enolpyruvate Inositol CH OH 2 CH OH 2 HOCH CH OH 2 CH O 2 HOCH P Glycerol UDP-Galactose UDP-Sugars CH CH OH 3 2 ETHANOL Dihydroxy- acetone-P (Glycerone-P) Glucosamine-6-P Phospho- serine Chain elongation Mitochondrial Serine Serine Indole-3-glycerol-P 3-Deoxy-D-arabino- heptulosonate-7-P Indoleacetate (Auxin) Indoxyl Formylkynurenine Kynurenine 3-Hydroxy kynurenine 3-Hydroxy anthranilate 2-Amino-3-carboxy muconate semialdehyde NH OH Tryptamine 1-(o-Carboxy phenylamino) 1-deoxyribulose-5-P N-(5-P-Ribosyl) anthranilate Anthranilate Catechol NH Indole- acetaldehyde NH CH CHO 2 Indolepyruvate N H C-CH(OH)CH(OH)CH O 2 P CH 2-Aminomuconate- 6-semialdehyde CH CH NH 2 2 2 NH 1.2.3.7 4.1.1.43 3.5.1.9 3.7.1.3 4.2.1.20 1.13.11.6 4.1.1.45 2.4.2.18 4.2.3.5 4.1.1.48 1.14.13.9 4.2.3.4 Dehydro- quinate 4.2.1.10 1.1.1.25 2.5.1.19 Shikimate-3-P Shikimate-5 enolpyruvate 3-P Chorismate PEP 2.7.1.71 1.4.1.19 Homogentisate Phenylpyruvate Cinnamate Coumarate Prephenate 2-Amino muconate Fumarate Aspartate GLYCINE Sarcosine Hydroxy- pyruvate P-Hydroxy- pyruvate SERINE Imidazole glycerol-P P-Ribosyl-ATP P-Ribosylformimino 5-aminoimidazole- carboxamide-R P Formimino glutamate Imidazolone propionate Urocanate Cystathionine Homocysteine Phosphoadenylyl- sulphate (PAPS) CH (NH )COOH 2 3 + Histidinol-P Histidinol Histidinal 3.1.3.15 2.6.1.9 1.1.1.23 3.6.1.31 5.3.1.16 2.4.2.17 Asparagine ALANINE 3-Sulphinyl pyruvate Oxobutyrate 2-Aceto-2- hydroxy- butyrate O-Phospho- homoserine HO SCH CH NH 2 2 2 2 Hypotaurine HO SCH CH NH 3 2 2 2 Taurine Glutamate γ-Glutamylcysteine Glutathione Glycine Cysteine β-Alanine Cysteate S-Adenosyl homocysteine 4.2.3.1 1.1.1.86 4.2.1.9 1.1.1.86 4.2.1.9 2.6.1.32 4.1.3.18 S-Adenosyl methionine (SAM) 2-Methylaceto- acetyl-CoA 2-Acetolactate 2-3-Dihydroxy isovalerate 2-Oxo- isovalerate 3-Hydroxy- isobutyrate 3-Hydroxy- Isobutyryl-CoA Methyl acrylyl-CoA 2-Methyl-3- hydroxy- butyryl-CoA Tiglyl-CoA 2 Methylbutyryl- CoA 2:3-Di-OH- 3-methylvalerate 2-Oxo-3-methyl valerate Isobutyryl-CoA Cysteine sulphinate 2.5.1.48 2-Isopropyl- malate CH CH COCOO 3 2 + CH CH(NH )COO 3 3 CH CH(OH)COO 3 HO SCH COCOO 2 2 OCH COCOO 2 P OCH CH(NH )COO 2 3 P HOCH COCOO 2 HOCH CH(NH )COO 2 3 + + OH CH COCOO 2 COO OH OH O COO OH OH HO P COO OH OH O CH 2 P COO O-C-COO OH O OH OOC CH COCOO 2 N H HOC-CH(OH)CH(OH)CH 2 OP CH COO NH 2 COO OH NH 2 COO COO OCH OOCCH NHCH 2 3 CH (CH ) COS-ACP 3 2 14 CH (CH ) COSCoA 3 2 14 CH (CH ) CH=CHCO-S-ACP 3 22 CH CH=CHCO-S-ACP 3 CH CH(OH)CH COS-ACP 3 2 COSCoA Linoleate Oleoyl-CoA Stearoyl-CoA Dehydrostearoyl-CoA OH-Stearoyl-CoA Oxostearoyl-CoA Palmitoyl-ACP Palmitoyl-CoA ACYL-ACP 2, 3-Enoyl-ACP 3, 4-Decenoyl-ACP 2, 3-Decenoyl-ACP 3-OH-Acyl-ACP 3-Oxoacyl-ACP 3-Oxo-Decanoyl-ACP 3-Oxo-Hexanoyl-ACP 3-OH-Hexanoyl-ACP 2, 3-Hexenoyl-ACP Butanoyl-ACP Crotonoyl-ACP 3-OH-Butanoyl-ACP Acetoacetyl-ACP Hexanoyl-ACP 3-OH-Decanoyl-ACP Decanoyl-ACP Palmitoleoyl-ACP γ-Linolenate Arachidonate Leukotriene B4 COSCoA CO-S-ACP Thromboxane B2 HOOCCH CO-S-ACP 2 Malonyl-ACP HOOCCH CO-SCoA 2 Malonyl-Co-A CH CO-S-ACP 3 Acetyl-ACP CH O-CO-R 2 CH OH 2 R’-CO-OCH FATTY ACID ACYL-CoA (Cytosol) CH O-CO-R 2 CH O 2 R’-CO-OCH P Cardiolipin Phosphatidylglycerol Acetylcholine Glycerophosphocholine Lysolecithin Choline plasmalogen CDP-choline Choline-P Mevaldate CH (CH ) CH=CHCH(OH)CHCH OH 3 2 12 2 Dehydrosphingosine Sphingosine 4-Sphingenin Psychosine Acyl-CoA Acyl-CoA Cerebroside Galactose CH (CH ) CH=CHCH(OH)CHCH O- 3 2 12 2 NHCOR Isopentenyl-PP (C5) Dimethylallyl-PP (C5) Geranyl-geranyl-PP (C20) Farnesyl-PP (C15) Squalene (C30) Geranyl-PP (C10) Desmosterol Zymosterol Lanosterol CH C = CHCH O 3 2 PP CH 3 Ceramide Gangliosides NHCOR CH O 2 PP OPP CH 2 CHOLESTEROL CH C(OH)CH CH OH 3 2 2 CH COO 2 CH C(OH)CH CHO 3 2 CH COO 2 NADP + Dehydroascorbate OH H O HOCH 2 C C C C CO OH H OH OH H O HOCH 2 C C CO CO CO H NAD+ Pi ADP ADP Phytoene (C40) Lycopene (C40) β-CAROTENE (C40) Rhodopsin Metarhodopsin Retinoate trans-Retinal 11-cis-Retinol trans-Retinol (Vitamin A) Retinol esters Dark Light Ubiquinone (Coenzyme Q) Menaquinone Plastoquinone Phylloquinone (Vitamin K) Opsin CHO CH OH 2 CHO CH OH 2 CH 3 CH O 3 CH O 3 n O O O O Phytol (C20) α-Tocopherol (Vitamin E) Quinolinate Quinolinate- nucleotide Nicotinate- nucleotide Desamino-NAD 5-Hydroxy- tryptophan 5-Hydroxytryptamine (SEROTONIN) N-Acetyl-5-O-methyl-serotonin (MELATONIN) NH CH CH NHCOCH 2 2 3 HO NH CH CH NHCOCH 2 2 3 CH O 3 NAD(P) + NICOTINATE N-Acetyl-serotonin 2.4.2.19 Dopamine Dopa Dopaquinone THYROXINE MELANIN OH OH CHOHCH NHCH 2 3 OH OH CHOHCH NH 2 2 4-OH-3-Methoxy- phenylglycol CHOHCH NH 2 2 OH OCH 3 OCH 3 CHOHCH OH 2 OH OCH 3 4-OH-3-Methoxy- D-mandelate Cyclic AMP 2H-folate 4H-folate ATP Dephospho-Coenzyme A 4-P-Pantetheine 4-P-Pantothenylcysteine 4-P-Pantothenate Pantoate 6.3.2.1 1.1.1.169 3.5.1.22 2.7.1.33 NH N H O O O O CH OH 2 OH OCH 3 CH(OH)COO H 2 NH 3 + CH-COO C O O COO N Ribose- P N + COO NH CH COO 2 O OH CH O 2 P NHCOCH NH 2 2 OH Formyl glycinamide-R P Urea Formyl glycinamidine-R P Allantoate Allantoin URATE ADP Xanthine Hypoxanthine Inosine Adenosine Adenylo- succinate 5-Amino imidazole-R P 5-Amino-4-imidazole carboxylate-R P 5-Amino-4-imidazole (N-succinylcarboxamide)-R P 5-Aminoimidazole carboxamide-R P Formylamido- imidazole- carboxamide-R P H NCONH 2 2 HN HC 2 CHO NH RP NH C C C O C CO OC NH NH H N HN H C C O C CH OC NH N N HN Carbamoyl β-alanine Dihydro- uracil Uracil d-ADP d-ATP GTP GDP XANTHOSINE-P (XMP) TTP TDP β-Ureido isobutyrate Dihydro thymine Thymine d-UMP d-CMP d-CDP CDP Carbamoyl aspartate Dihydro orotate Orotate Orotidine-P Uridine-P (UMP) UDP 3-Amino- isobutyrate CH C NH 2 CH OC NH N CH C O CH OC N H HN CH-CH 3 C O OC NH HN CH 2 CH 2 CH 2 C O OC NH HN C O CH OC NH HN C CH 3 Methylmalonyl semialdehyde OHCCHCOO CH 3 CH 2 C O CH-COO OC NH HN CH C O C-COO OC NH HN H NCONHCH CH COO 2 2 2 C C C CH RP HC NH NH OOC-CH-CH COO 2 N N N CHO NH RP NH OC HC 2 2.7.7.43 3.1.3.29 1.1.1.158 5.1.3.13 3.1.3.29 4.1.3.20 2.7.1.60 5.1.3.14 5.1.3.7 5.1.3.1 2.7.1.4 5.4.2.3 3.1.1.17 1.1.1.49 1.1.1.21 2.7.7.23 3.1.3.25 1.13.99.1 1.1.1.19 1.1.3.8 5.3.1.3 2.7.1.53 1.1.1.9 2.7.1.15 2.7.1.17 5.1.3.4 5.1.3.1 2.2.1.2 4.1.2.13 2.7.1.11 2.7.1.47 2.2.1.1 1.2.1.12 1.1.1.29 1.1.1.95 1.3.1.35 1.14.19.3 2.3.1.41 2.3.1.41 2.3.1.41 1.1.1.100 5.3.99.5 4.2.1.60 5.3.99.3 1.14.99.1 1.13.11.34 1.3.1.10 1.3.1.9 1.14.19.1 1.3.1.9 4.2.1.61 4.2.1.60 4.2.1.60 4.2.1.59 4.2.1.58 1.3.1.9 1.3.1.9 6.2.1.3 3.1.2.20 1.1.1.100 5.3.1.1 HOOC-COOH Oxalate Glycolate HOCH CHO 2 Glycol aldehyde Ethanolamine-P 2.7.1.30 2.3.1.7 3.7.1.2 2.7.8.8 2.1.1.17 2.1.1.71 1.3.1.35 2.7.8.2 3.1.4.3 3.1.4.4 2.7.7.15 3.1.1.5 3.1.4.2 2.7.1.32 2.7.1.82 1.2.4.1 2.3.1.12 1.8.1.4 2.6.1.2 1.4.1.1 4.1.1.1 2.3.1.50 1.1.1.102 2.7.8.3 2.4.1.62 3.2.1.46 5.2.1.3 1.2.1.36 2.3.1.76 3.1.1.21 5.2.1.7 5.4.99.7 1.14.99.7 4.2.1.75 1.3.3.3 4.99.1.1 2.5.1.29 4.1.1.28 2.3.1.5 2.1.1.4 6.3.5.1 6.3.1.5 2.4.2.11 2.7.7.18 2.6.1.5 1.2.1.32 1.13.11.5 2.1.1.28 2.1.2.2 6.3.3.1 3.5.2.5 1.4.1.10 1.7.3.3 1.1.3.22 4.1.1.28 4.1.1.21 2.1.2.3 2.4.2.1 1.5.99.2 2.1.1.5 2.6.1.22 3.5.2.3 2.6.1.51 1.4.1.7 3.1.3.3 2.6.1.52 2.1.3.2 ACETATE Glycine 2.4.2.4 2.1.1.45 RP CH C O CH OC N HN 1.3.1.14 2.4.2.10 4.1.1.23 2.7.7.7 2.7.7.7 2.7.7.7 1.17.4.1 2.7.7.7 2.7.4.14 4.4.1.1 1.8.1.6 1.13.11.20 4.4.1.8 4.2.1.22 1.1.1.27 4.1.1.29 6.3.2.3 6.3.2.2 1.8.1.3 LACTATE Acetaldehyde 2.7.1.24 2.7.7.3 4.1.1.36 Bile Acids CH CH COSCoA 3 2 Propanoyl-CoA 1.1.1.31 2.1.3.1 4.1.1.41 5.1.99.1 6.3.2.5 2.1.1.14 2.1.1.13 4.6.1.1 CDP-Ethanolamine 1.1.1.35 4.2.1.17 + HO SCH CH(NH )COO 2 2 3 3.5.2.7 NH HN CH OOC CHCH CH COO 2 2 CMP-N-Acetyl neuraminate CDP-diacyl glycerol CHOLINE Ethanolamine Glyoxylate HISTAMINE 2.3.1.46 Homoserine Tyramine Plant Pigments Tannins Maleyl acetoacetate Fumaryl acetoacetate 5.2.1.2 CH COO 2 OH OH OH CH CH NH 2 2 2 OH OH CH CH NH 2 2 2 Hydroxyphenyl pyruvate α-Tocopherol (Vitamin E) LIGNIN d-GTP OH OH OH O HO OH P OPPT CH 3 HO OH OH O NADPH 2.2.1.1 2.7.1.3 1.1.1.45 1.1.1.130 1.10.3.3 1.10.2.1 1.1.1.10 ATP CH CH NH 2 2 2 NH HO 5.4.99.5 2.6.1.5 1.13.11.27 Indole 4.1.99.1 1.13.11.11 OH O OH NH C C CH CH O 2 P H H COO NH 2 COO 6.3.5.3 6.3.2.6 4.3.2.2 Guanine DNA 6.3.4.4 4.3.2.2 C C O C CH HC NH N N HN C C O C CH HC NH N N HN 3.5.3.4 2.4.2.1 2.7.7.6 2.7.7.6 2.7.7.6 2.7.7.6 2.7.4.6 2.7.4.3 2.7.4.4 2.7.4.6 2.4.2.15 1.1.1.205 6.3.4.1 6.3.5.2 2.7.4.4 3.5.4.3 3.2.2.2 2.4.2.1 3.1.3.5 3.1.22.1 6.3.4.2 1.17.4.1 1.17.4.1 3.5.4.12 2.7.4.8 2.7.4.9 2.7.4.6 2.4.2.4 1.3.1.2 3.5.2.2 3.5.1.6 3.5.1.6 3.5.2.2 3.5.4.1 Diphospho- mevalonate Mevalonate 3-Oxopentanoyl-CoA 3-Oxoacyl-CoA 3-OH-Acyl-CoA 2, 3-Enoyl-CoA 2, 3-Hexenoyl-CoA 3-OH-Hexanoyl-CoA Acetoacetyl-CoA 3-OH-Butanoyl-CoA Crotonoyl-CoA Pentanoyl-CoA 3-OH-Pentanoyl-CoA 1.1.1.35 1.1.1.35 1.1.1.157 1.3.99.3 1.3.99.2 1.1.1.35 Odd C Fatty acids Succinylhomoserine CO 2 2-OXO ACID Glutamyl-P Glutamine UREA P-Creatine Creatine Creatinine Glycine CITRULLINE Glutamic semialdehyde 2-AMINO ACID Pyrroline-5- carboxylate Glyoxylate Pyruvate 4-Hydroxy- 2-oxoglutarate 4-Hydroxy- glutamate 3-Hydroxy- pyrroline- 5-carboxylate Putrescine H NCH CH CH CH NH 2 2 2 2 2 2 Spermidine Spermine N -Trimethyllysine 6 6 N -Trimethyl- 3-OH-lysine Carnitine Glutaryl-CoA Saccharopine 2-Aminoadipate semialdehyde 2-Aminoadipate 2-Oxoadipate Arginino- succinate Guanidoacetate Methylmalonyl-CoA 4.1.2.12 Aspartyl Semialdehyde 2, 3-Dihydro- dipicolinate 1.2.1.11 4.2.1.52 1.3.1.26 Piperideine- 2, 6-dicarboxylate N-Succinyl- 2-amino-6-oxo- pimelate 2.6.1.17 N-Succinyl-2, 6 diaminopimelate Diamino- pimelate 3.5.1.18 3-Methyl- glutaconyl-CoA (CH ) CHCH COSCoA 32 2 (CH ) CHCH COSCoA 32 2 3-Methyl- crotonyl-CoA Isovaleryl-CoA Oxopantoate HCHO OOCCH C = CHCOSCoA 2 CH 3 CH COCHCOSCoA 3 CH 3 CH CH=CHCOSCoA 3 CH 3 CH CH(OH)CHCOSCoA 3 CH 3 C(OH)CH(OH)COO CH CH 3 2 CH 3 CH = CCOSCoA 2 CH 3 HOCH CHCOS-CoA- 2 CH 3 CH CHCO-SCoA 3 CH 3 C (OH)CH(OH)COO CH 3 CH 3 CH CH CHCOSCoA 3 2 CH 3 CH C = CHCOSCoA 3 CH 3 ATP 4-Aminobutyrate (GABA) 1.3.99.7 1.5.1.2 1.14.11.2 2.6.1.39 1.2.1.31 1.14.11.8 2.5.1.16 2.5.1.22 4.1.3.16 2.6.1.23 1.5.99.8 1.5.1.2 6.4.1.4 4.2.1.33 2.6.1.6 1.1.1.85 Oxoleucine COOH (CH ) CHCHCH(OH)COO 32 S-Adenosylmethyl thiopropylamine (Decarboxylated SAM) PROLINE Coenzyme A CH C= CHCH 3 2 CH 3 O CH CH 3 3 HO CH 3 CH 3 CH 3 CH C-CH H O 3 2 2 C PP CH 2 CH C= CHCH O 2 2 PP CH 3 3-P-Glycerol Phosphatidyl ethanolamine CEPHALIN 3.1.4.12 Progesterone 1.5.1.12 2.7.3.2 4.2.1.17 3.1.2.4 2.6.1.32 4.4.1.15 Acetylserine 2.7.7.4 2.7.1.25 1.8.99.1 Adenylylsulphate (APS) 2.7.1.39 4.4.1.15 4.1.1.29 2.5.1.47 2.3.1.30 P-Ribosyl-PP 1.1.1.1 1.2.1.4 Aspartyl-P 5.1.1.7 2.7.1.40 2.2.1.6 4.1.3.18 1.5.1.9 1.14.11.1 CHOLINE 2.1.1.13 1.3.1.2 NHCH CH SH 2 2 NHCH CH CO 2 2 OCH C(CH ) CH(OH)CO 2 32 P ADP- NHCH CH SH 2 2 NHCH CH CO 2 2 OCH C(CH ) CH(OH)CO 2 32 P-ADP- NHCH CH SH 2 2 NHCH CH CO 2 2 OCH C(CH ) CH(OH)CO 2 32 1.4.1.8 1.3.99.3 1.3.99.3 1.4.1.9 4.2.1.18 4.1.1.20 1.3.99.7 1.8.99.2 + CH CO-OCH CH(NH )COO 2 2 3 2.5.1.6 2.1.1.10 2.1.1.20 1.1.1.3 1.1.1.3 4.2.1.18 4.1.2.5 4.3.1.5 4.2.1.20 2.1.1.20 Glyoxylate 2.1.1.6 1.4.3.4 Hexanoyl-CoA Butanoyl-CoA 6.3.5.5 2.7.2.11 NO 1.14.13.39 3.5.3.6 3.5.3.1 4.1.1.17 2.7.7.41 2.6.1.4 1.1.1.100 1.1.1.8 2.3.1.51 2.3.1.15 2.7.8.5 SO - 4 2 1.14.16.4 NH 2 OOC OOC 4.1.1.25 1.3.1.13 1.3.1.13 2.7.4.6 CYTIDINE- triphosphate (CTP) Cytosine 2.4.2.9 4.1.1.11 2.6.1.18 4.3.1.3 4.2.1.49 3 HSO - 1.1.1.105 2.3.1.41 NADPH 1.1.1.22 2.3.1.4 5.5.1.4 3.2.1.26 3.2.1.48 2.6.1.16 1.1.1.14 2.7.7.34 4.2.1.47 2.4.1.68 2.4.1.69 2.4.1.9 5.3.1.8 2.4.1.11 O OPPU CH OH 2 CH OH 2 HO OH OH HO 2.4.1.21 4.2.1.52 1.2.1.18 4.2.1.18 O CH C CH OC N HN DP CH C O CH OC N HN RPPP OCH 2 H C C H OH OH CH N HN C H C P H O OCH 2 C C H OH H OH C H C C C NH 2 C CH HC N RP(PP) N N N + P NH 2 H O OCH 2 C C H OH H OH C H C C OC C CH HC N RP N N NH P H OCH 2 C C H OH OH CO CH 2 C CONH 2 C CH HC N RP N N NH P + OOCCH N(CH ) 2 33 Betaine Betaine aldehyde 2.4.1.16 5.1.3.6 2.4.1.33 2.7.7.13 5.4.2.8 5.3.1.8 O OH CH OH 2 HO O P HO 2.7.1.28 2.7.1.31 6.3.4.13 5.3.1.1 2.2.1.1 4.1.1.9 3.1.2.11 β-OH-β-Methyl- glutaryl-CoA 6.3.4.5 5.3.1.6 2.5.1.21 2.1.1.2 3.5.2.10 6.3.2.13 6.3.2.7-10 2.7.7.27 2.7.7.9 3.5.4.10 C C O C CH OC N RP N N H HN C C O C CH N N N HN RP HN 2 C 2.7.4.6 1.17.4.1 2.7.4.6 2.6.1.13 Carbamoyl-P 2.3.1.9 Carnitine 2.6.1.4 2.6.1.44 CH C(OH)CH COSCoA 3 2 CH COO 2 1.2.3.5 1.1.1.34 2.3.1.16 4.1.3.5 2.3.1.16 Menaquinone 4.1.1.15 4.3.1.19 Carnosine CH CH NH 2 2 2 C NH N C HC H 4.1.1.22 4.3.1.3 Ubiquinone Plastoquinone D-Ribose-5-P NH CH CH(NH )COO 2 3 HO + O OH OH HO OPPU COO - O OH OH HO OPPU COO - OH H OH HOCH 2 C C CO H CO COO - O CHOH CHOH AcNH HO OPC O CO - CH OH 2 O CHOH CHOH AcNH HO OH COO CH OH 2 O OH OHHO HO CH O 2 P O OH ACNH HO OH CH O 2 P O O OH HO HO P CH OH 2 O OH OH HO HO CH OH 2 O OH HO OH 3 CH O 2 P O OH HO O NHCOCH 3 P CH OH 2 CH OH 2 CH OH 2 O OH HO OH NH 2 CH O 2 P O OH HO OH OPPU CH OH 2 O O HO OH OH HO P CH OH 2 CH OH 2 O O HO OH OH O OH OH OH CH OH 2 O OH HO OH OH CH 2 CH OP 2 OH H H H OH C C C C OH OH H COO - HOCH 2 H OH OH OH C C C CHO H H HOCH 2 H OH OH H C C C CHO H OH HOCH 2 OH H OH H C C C CHO H OH HOCH 2 H OH H OH C C C CHO OH H HOCH 2 OH H H OH C C C CHO OH H HOCH 2 OH H OH OH C C C CHO H H HOCH 2 H OH H OH C C C C OH H H OH COO - POCH 2 OH H OH C C CO H POCH 2 CH OH 2 H OH H C C CO OH HOCH 2 CH OH 2 CH OH 2 POCH 2 H OH OH C C CO H CH OH 2 POCH 2 OH H H C C CO OH CH OH 2 POCH 2 H OH OH C C CO H HOCH 2 CH OH 2 OH H OH C C CO H HOCH 2 CH OH 2 OH H H C C CO OH HOCH 2 CH OH 2 H OH OH H C C C H OH HOCH 2 CH OH 2 OH H OH OH C C C H H HOCH 2 CH OH 2 H OH H OH C C C C OH H H OH CH OH 2 HOCH 2 H OH OH C C CHO H POCH 2 H OH H OH C C C CO OH H HOCH 2 CH O 2 P H OH OH OH C C C H H HOCH 2 CH OH 2 H OH OH C C C H CO H HO POCH 2 CH OH 2 P H OH OH C C C H CO H HO CH O 2 P OCH 2 H OH OH C C H C CHO OH H POCH 2 POCH 2 O OH NH 2 OH CH O 2 P CHOHCHO POCH 2 HOCH 2 COCH 2 OP O OH OO P P OH CH O 2 P CHOHCOOP POCH 2 CHOH POCH 2 COO COO OH O HO OH COO CH (CH ) CH(OH)CH COS-CoA 3 2 14 2 CH (CH ) CH(OH)CH COS 3 2 14 2 CH (CH ) COCH COS-CoA 3 2 14 2 CH (CH ) COCH COS-CoA 3 2 14 2 CH (CH ) COS-CoA 3 2 n+2 CH (CH ) CH=CHCOS-CoA 3 2 14 CH (CH ) 3 2n CH=CHCOS-CoA CH (CH ) 3 25 2 CH=CHCH COSACP CH (CH ) 3 26 CH=CHCOSACP CH (CH ) 3 26 COCH COSACP 2 CH (CH ) 3 26 2 2 CH CH COSACP CH (CH ) 3 22 2 2 CH CH COSACP CH CH CH COSACP 3 2 2 CH (CH ) 3 26 CH(OH)CH COSACP 2 CH CH=CHCO.S-ACP 3 CH (CH ) 3 22 2 COCH COSACP CH 3 COCH COSACP 2 CH (CH ) 3 2n 2 COCH COSACP CH (CH ) 3 2n 2 CH(OH)CH COSACP CH (CH ) 3 22 2 CH(OH)CH COSACP ACYL-CoA (Mitochondria) CH (CH ) CH CH COSCoA 3 2 2 2 n CH (CH ) CH=CHCOSCoA 3 2 n CH (CH CH(OH)CH COSCoA 3 2 n CH (CH ) CH CH COSCoA 3 22 2 2 CH (CH ) CH=CHCOSCoA 3 22 CH (CH ) COCH COSCoA 3 2 2 n CH COCH COSCoA 3 2 CH (CH ) CH(OH)CH COSCoA 3 22 2 CH CH(OH)CH COSCoA 3 2 CH CH=CHCOSCoA 3 CH CH CH COSCoA 3 2 2 CH CH CH CH COSCoA 3 2 2 2 Pentenoyl-CoA CH CH CH=CHCOSCoA 3 2 CH CH CH(OH)CH COSCoA 3 2 2 CH (CH ) COCH COSCoA 3 22 2 CH CH COCH COSCoA 3 2 2 OH OH HO OH O O- CH O-PO 2 CH CHOHCH OH 2 2 O CH 2 CH 2 R'-CO-OCH O-CO-R O-PO O- HOCH CH 2 2 NH 3 + POCH CH 2 2 NH 3 + CPP- OCH CH 2 2 NH 3 + O - CH O-CO-R 2 CH O P 2 OCH CH 2 2 NH 3 + CH COCH CH N(CH ) 3 2 2 33 HOCH CH N(CH ) 2 2 33 + CH CH 2 COO (NH ) 3 + OH OH CH CH 2 COO- (NH ) 3 + CO NH 2 CH CH(NH )COO 2 3 + NH OC CO NH OC N H C H HN 2 HC CH N N C RP HN 2 CH C CH OC N N DP NH 2 RPPP CH C CH OC N N NH 2 C C C CH RP(P) HC N N N N NH 2 H NCOO 2 P (CH ) NH 23 2 H N(CH ) NH 2 23 (CH ) NH 24 (CH ) NH 23 2 H N(CH ) NH 2 24 CH -SCH CH CHNH 3 2 2 2 Adenosyl + + CH 3 CH 3 CHCH(NH )COO 3 + C NH N C HC H CH CH(NH )CH OH 2 3 2 + C NH N C HC H CH CH(NH )CH OP 2 3 2 C NH N C H HC CH COCH OP 2 2 + HSCH CH CH(NH )COO 2 2 3 + + SCH CH CH(NH )COO 2 2 3 CH CH(NH )COO 2 3 RP C O C-COO OC N HN CH 2.4.1.23 CH (CH ) CH=CHCH(OH)CHCH OH 3 2 12 2 NH 3 + CH (CH ) CH(OH)CHCH OH 3 2 14 2 NH 3 + CH (CH ) COCHCH OH 3 2 14 2 NH 3 + Galactose CH (CH ) CH=CHCH(OH)CHCH O- 3 2 12 2 NH 3 + 4.1.1.70 H NCONH 2 2 OHCCH N(CH ) 2 33 + C-CH3 C O CH OC N DP HN DP CPP-OCH CH N(CH ) 2 2 33 + OCH CH N(CH ) 2 2 33 + 1.2.1.41 UDP-N-Ac-Muramate O O CH CH 3 COO- NHAC HO OPPU CH OH 2 2.5.1.57 O OH CH 3 HO HO OPPG 2.7.7.24 2.4.1.22 2.7.7.12 5.1.3.2 5.3.1.4 Sedoheptulose-PP 3.1.1.28 4.2.1.24 N CH 2 CH 2 N N N CH 3 CH 3 CH CH COO CH CH CH 2 HC 3 HC 3 HC 3 HC C C H H Fe CH 2 CH 2 COO - - CH 3 CH 3 CH 2 CH 2 CH 2 HC 3 HC 3 N H N H N H N H CH CH HC 2 C C H 2 H 2 - CH 2 CH 2 COO CH 2 CH 2 COO - HC 2 HC 2 CH 2 CH 2 OOC COO HN 2 N H - - CH 2 HC 2 HC 2 HC 2 H 2 H 2 CH 2 CH 2 CH 2 CH 2 CH 2 CH 2 N H N H N H N H COO COO COO COO C C - - CH 2 CH 2 COO CH 2 CH 2 COO - - - - OOC - OOC - CH 3 CH 3 HC 3 HC 3 HC 2 H 2 H 2 N H N H N H N H CH 2 CH 2 CH 2 CH 2 CH 2 C C COO - CH 2 CH 2 COO CH 2 CH 2 COO - - COO - COO - CH C(OH)CH CH O 3 2 2 PP CH COO 2 OHCCOO HOCH COO 2 NH 2 H NCNHCH COO 2 2 + NH 2 H NCN(CH )CH COO 2 3 2 + NH 2 HNCN(CH )CH COO 3 2 P- + HN C NH CO N CH2 CH 3 OOCCHCH COO 2 H NCNHCH CH CH CH 2 2 2 2 N (NH ) 3 + COO CHCOO HC CH 2 HOCH N OOCCH(OH)CH CH(NH )COO 2 3 + CHCOO CH 2 HOCH HC 2 N H NH CHCOO CH 2 CH 2 CH 2 OOCCH(OH)CH COCOO 2 N CHCOO CH 2 CH 2 CH CH COCOO 3 OHCCOO + H NCONHCH CH CH CH 2 2 2 2 (NH ) 3 COO H NOCCH CH CH 2 2 2 COO (NH ) 3 + R-CO-COO + COO R-CH(NH ) 3 + COO H NOCCH CH 2 2 (NH 3 + OOC-CH-COSCoA CH 3 P OOCCH CH 2 COO (NH ) 3 + OOCCH CH CHO 2 2 OHCCH CH 2 COO (NH ) 3 + (CH ) NCH CH(OH)CH COO 33 2 2 + OHCCH CH CH 2 2 + COO (NH ) 3 POOCCH CH CH 2 2 + COO (NH ) 3 OHCCH CH CH CH 2 2 2 COO (NH ) 3 + NH CHCH CH COO 2 2 COO CH CH CH CH CH 2 2 2 2 COO (NH ) 3 + H N(CH ) 2 4 2 CH(NH )COO 3 CH(NH 3 + (CH ) N(CH ) 33 23 CH CH(NH )COO 2 3 + + CH 2 C H OOCC N HC CH-COO CH 2 C H 2 CH-COO OOCC N HC 2 OOCCOCH CH CH CH-COO 2 2 2 CH CH 2 2 2 OOCCH CH CONH 2 2 OOCCHCH CH CH CH-COO 2 2 2 OOCCH CH CONH 2 2 CH 2 2 NH 3 + OOCCH-CH CH CH CHCOO 2 2 2 NH 3 + NH 3 + (CH ) CHC(OH)CH 32 2 COOH COO (CH ) CHCH COCOO 32 2 + (CH ) CHCH 32 2 CH(NH )COO 3 HOCH CHCOO 2 CH 3 CHCOCOO CH 3 CH 3 CH COC(OH)CH 3 3 COO + POCH CH CH(NH )COO 2 2 3 + HOCH CH CH(NH )COO 2 2 3 + CH CH CH(NH )COO 2 2 3 OOCCH CH 2 COO 2 + CH SH 2 OOCCH(NH )CH CH CONHCHCONHCH COO 3 2 2 2 HOCH C(CH ) COCOO 2 32 HOCH C(CH ) CH(OH)COO 2 32 NHCH CH COO 2 2 HOCH C(CH ) CH(OH)CO 32 ) 2 32 NHCH CH COO 2 2 OCH C(CH ) CH(OH)CO 2 32 P CH CH 3 2 CH 3 CH + CH(NH )COO 3 CH COC(OH)CH CH 3 2 3 COO CHCOCOO CH CH 3 2 CH 3 NHCHCH SH 2 COO NHCH CH CO 2 2 OCH C(CH ) CH(OH)CO 2 32 P + CH SH 2 OOCCH(NH )CH CH CONHCHCOO 3 2 2 + Adenosyl SCH CH CH(NH )COO 2 2 3 + SCH CH CH(NH )COO 2 2 3 + HO SCH CH(NH )COO 3 2 3 + + S-CH CH(NH )COO 2 3 S-CH CH(NH )COO 2 3 + HSCH CH(NH )COO 2 3 1.1.1.23 H C NH N C HC CH CHCOO 2 NHCOCH CH NH 2 2 2 + H C NH N C HC CH CH(NH )COO 2 3 CHCH CH COO 2 2 NH N CH OC C CH CHCOO NH N CH CH CH COO 3 N H OOC CH-COO OC NH 2 CH 2 H NCH CH COO 2 2 2 H NCH 2 2 CH 3 CHCOO H NCONHCH CHCOO 2 2 CH 3 C C O CH RP HC N N N HN C C C O CH HCO N N N RP C CH N N HN 2 H C C C O CH N N C C CH N N RP HN 2 HN 2 C O O O P~ ~ P O O O O PO O CH2 N N N N O OH OH NH 2 HC CH N N N N O O P O O O CH2 OH NH 2 HC CH COO CO NH OC N H C H HN 2 NH 2 OOC-CH-CH COO 2 HNCO C CH N N C RP RP HN 2 OOC C CH N RP N C HN 2 CH=CHCOO OH CH=CHCOO CH COCOO 2 CH 2 P COO OH O O O COO O CH COO 2 O CH COO 2 -OOC OCH 2 CH 2 OH H C P HOCH HCOH OC COO COO HO OH OH O NH 2 COO OCH NH CH COCOO 2 NH CHO CO NH 2 OH CH CH(NH )COO 2 3 + N COO COO N + COO COO RP O OH OH HO OPPU COO O OH NHCOCH 3 HO OPPU COO CH OH 2 O O C CH 2 NHAC HO OPPU COO CH OH 2 O OH HO OH OH COO OOCCH N(CH ) 2 32 Dimethylglycine 1.1.99.1 1.2.1.8 COO HO HO COO O HO OH COO CH(O ) P POCH2 COO CH(OH) HOCH 2 COO COO CH(O ) P HOCH 2 COO CH =C(O ) 2 P COO CONH 2 Ribose -O - P - O - P - O- Adenosine(P) + N O O O O + COO N Ribose - O - P - O - P - O -Adenosine O O O O I I I I OH O CH CH 2 COO (NH ) 3 + Epinephrine (Adrenaline) Norepinephrine (Noradrenaline) Normetepinephrine (Normetadrenaline) HOCH CH N (CH ) 2 2 33 + Adenosyl + + SCH CH CH(NH )COO 2 2 3 CH 3 (CH ) N(CH ) 33 23 CH CH(NH )COO 2 3 + + OH OOCCH CH CH COSCoA 2 2 2 OOCCH CH CH NH 2 2 2 2 OOCCH CH CH COCOO 2 2 2 OOCCH CH CH CH 2 2 2 COO COO (NH ) 3 + HO H HO H H HO H HO H Prostaglandin PGE 2 OH HOCH 2 C C CO C COO OH H OH H H - COO O R'-CO-OCH CH O-CO-R 2 CH O POCMP 2 O CH CH N(CH ) 2 2 3 + O HOCH CH OPO 2 CH OH 2 O- CH OPO 2 O CH OCH=CHR 2 CH CH N(CH ) 2 2 33 + O- CH OPO 2 O R'-CO-OCH CH O-CO-R 2 3.1.1.32 CH CH N(CH ) 2 2 33 + O- O HOCH CH OPO 2 CH O-CO-R 2 CH CH N(CH ) 2 2 33 + O- CH CH(NH )COO 2 3 + + C NH N C HC H CH CH(NH )CHO 2 3 HS 11-cis-Retinal Pi ADP NADP + GDP- Mannuronate Mannose-6-P Dehydro- shikimate Shikimate RNA d-GDP d-CTP GUANOSINE-P (GMP) β-Alanine P-Ribosyl-AMP P-Ribulosylformimino 5-aminoimidazole- carboxamide-R P Imidazole acetol-P HS HSO - PANTOTHENATE 3-Isopropyl- malate Succinic semialdehyde 2.4.1.17 Diacyl glycerol 2.3.1.6 1.14.12.1 1.4.4.2 CH CHO 3 3.5.4.19 4.2.1.19 3.3.1.1 4.1.1.12 4.1.2.5 COO- 2 OHCCH 2.1.4.1 2.4.99.7 5.1.3.12 1.1.1.132 4.2.1.46 2.7.1.7 2.4.1.29 2.4.1.21 2.4.1.13 5.4.2.2 2.7.1.6 2.7.7.10 3.1.1.17 4.1.1.34 2.7.1.47 2.7.1.16 ADP P-Glucono lactone 1.1.1.44 O O OH HO OH CH O 2 P PHENYLALANINE TYROSINE TRYPTOPHAN INOSINE-P (IMP) THYMIDINE-P HISTIDINE URIDINE- triphosphate (UTP) CYSTINE CYSTEINE ISOLEUCINE LEUCINE LYSINE ORNITHINE ARGININE HYDROXY PROLINE PHOSPHATIDYL SERINE Phosphatidyl inositol LECITHIN SPHINGOMYELIN STEROIDS Pregnenolone 2.7.6.1 2.4.2.14 6.3.4.7 5.3.1.9 3.1.3.11 1.2.1.13 2.7.2.3 H OH OH C C C H CO OH C H H OH POCH 2 CH O 2 P 5.4.2.1 ADP 4.2.1.11 Endoplasmic Reticulum 1.1.1.100 3.1.1.3 Phosphatidate 2.3.1.39 4.2.1.17 4.2.1.17 4.2.1.55 1.1.1.79 1.2.1.21 1.4.3.8 1.1.1.32 2.7.1.36 2.7.4.2 4.1.1.33 2.5.1.1 2.5.1.10 2.7.8.5 2.7.8.1 4.1.1.65 2.7.7.14 1.3.99.7 PO O CH (CH ) CH=CHCH(OH)CHCH O 3 2 12 2 NHAcyl CH CH N(CH ) 2 2 33 + O- 3.5.1.23 1.14.99.36 2.5.1.32 1.1.1.105 2.4.2.19 1.2.1.32 METHIONINE 4.1.3.27 4.2.1.51 1.14.17.1 1.14.18.1 1.14.13.11 Glycinamide- ribosyl-P 4.2.1.22 3-Oxohexanoyl-CoA THREONINE 1.5.1.7 - 10 GLUTAMATE 2.3.1.38 ATP NADH SUCROSE ADENOSINE-P (AMP) Triacylglycerol FAT RNA CH 3 CH 2 OC-COO OH COO CH CH 2 COO (NH ) 3 + GLYCOPROTEINS GANGLIOSIDES MUCINS 4.1.2.- 2.6.1.- GLUCOSE O-Acyl-carnitine O-Acyl-carnitine OH O - - R-CO-OCH R'-CO-OCH R'-CO-OCH O HCO-CO-R CH CH(OH)CH O-P-OCH 2 2 2 CH O-CO-R’ 2 O R'-CO-OCH O- CH O-CO-R 2 CH O-PO 2 O- 2.7.8.11 H NCH CH CH CH 2 2 2 2 COO (NH ) 3 + 2.3.1.20 3.1.3.4 2.7.1.107 NH 2 + + H NCNHCH CH CH CH 2 2 2 2 COO (NH ) 3 H OH H OH C C C CO OH H HOCH 2 CH OH 2 R-CH 2 COO CH O-CO-R 2 CH O-CO-R" 2 R’-CO-OCH OH O CH CHNH 2 3 + O R'-CO-OCH O- CH O-CO-R 2 CH O PO 2 COO OOCCH CH CH 2 2 COO (NH ) 3 + CH CH(OH)CH(NH )COO 3 3 O OH HO OH OH CH OH 2 CH OH 2 NH CH O-CO-R 2 1.3.1.13 4.1.1.28 Malonic semi- aldehyde 2.7.2.4 2.1.3.3 3.5.1.2 6.3.1.2 6.3.5.5 Carbohydrates Biosynthesis Degradation Amino Acids Biosynthesis Degradation Lipids Biosynthesis Degradation Purines & Pyrimidines Biosynthesis Degradation Biosynthesis Degradation Vitamins Co-enzymes & Hormones Pentose Phosphate Pathway Photosynthesis Dark Reactions COMPARTMENTATION The "Backbone" of metabolism involves GLYCOLYSIS in the CYTOPLASM, the TCA CYCLE (mainly) in the Mitochondrial matrix and ATP FORMATION spanning the MITOCHONDRIAL INNER MEMBRANE An electron flow (an electric current) generated from NADH and UQH 2 drives the translocation of protons from the matrix to the intermembrane space. The retrolocation of these protons through the F0 subunits of ATP synthase to the matrix then supplies the energy needed to form ATP from ADP and phosphate Electron Flow Proton Flow Small Numbers ( eg. 2.4.6.7) refer to the IUBMB Enzyme Commission (EC) Reference Numbers of Enzymes LEGEND Human Metabolism is identified as far possible by black arrows Biosynthesis Degradation HEMOGLOBIN CHLOROPHYLL ATP TR A N S L O C A T E D PROTONS 2.7.1.1 ATP 2.7.1.2 3.7.1.2 2.4.1.1 3.2.1.33 3.2.1.23 3.2.1.108 2.7.1.6 2.7.7.12 3.1.3.9 3.2.1.10 2.4.1.25 2.4.2.8 3.5.4.4 1.5.1.3 1.4.4.2 2.6.1.44 4.1.3.4 1.9.3.1 2. 5.1. 61 4.1.1.37 1.3.3.4 1.3.99.3 3.2.1.45 3.2.1.52 3.1.4.12 1.14.16.1 1.14.18.1 1.17.3.2 4.2.1.22 6.4.1.3 1.2.1.24 2.6.1.19 6.3.4.16 2.1.3.3 4.3.2.1 6.3.4.5 5.4.99.2 1.3.99.10 1.2.4.4 1.2.4.4 1.2.4.4 Inborn Errors of Metabolism AMINO ACID METABOLISM Fumarylacetoacetase (3.7.1.2) Tyrosinaemia Type I MIM 276700 Phenylalanine hydroxylase (1.14.16.1) Phenylketonuria (PKU) MIM 261600 Tyrosinase (1.14.18.1) Tyrosine Negative Albinism MIM 203100 Glycine dehydrogenase (1.4.4.2) Non Ketotic Hyperglycinaemia (NKH) MIM 238300 PURINE & PYRIMIDINE METABOLISM Xanthine oxidase (1.17.3.2) Xanthinuria MIM 278300 Hypoxanthine guanine phospho-ribosyl transferase (2.4.2.8) HGPRT Deficiency, Primary Gout, Lesch-Nyhan Syndrome MIM 308000 Adenosine deaminase (3.5.4.4) Severe Combined Immuno-Deficiency (SCID) MIM 102700 FOLIC ACID Dihydrofolate reductase (1.5.1.3) Megaloblastic Anaemia MIM 126060 ORGANIC ACIDURIAS Propionyl-CoA carboxylase (6.4.1.3) Propionic Acidaemia MIM 606054 Methylmalonyl-CoA mutase (5.4.99.2) Methylmalonic Acidaemia MIM 251000 Isovaleryl-CoA dehydrogenase (1.3.99.10) Isovaleric Acidaemia MIM 243500 AMINO ACID METABOLISM Cystathionine synthetase (4.2.1.22) Homocystinuria MIM 236200 Branched chain ketoacid decarboxylase (1.2.4.4) Maple Syrup Urine Disease MIM 248600 GABA METABOLISM NEUROTRANSMITTER DEFECTS Succinic semialdehyde dehydrogenase (1.2.1.24) 4-Hydroxybutyric Aciduria MIM 271980 4-Aminobutyrate aminotransferase (2.6.1.19) GABA Transferase Deficiency MIM 137150 UREA CYCLE DEFECTS (HYPERAMMONAEMIA) Carbamoyl phosphate synthase (6.3.4.16) CPS Deficiency MIM 237300 Ornithine carbamoyl transferase (2.1.3.3) OCT Deficiency MIM 311250 Argininosuccinate synthase (6.3.4.5) Citrullinaemia MIM 215700 Argininosuccinate lyase (4.3.2.1) Argininosuccinate Aciduria MIM 207900 GLYCOGEN STORAGE DISEASES MUSCLE GLYCOGENOSES Muscle glycogen phosphorylase (2.4.1.1) GSD Type V McArdle Disease MIM 232600 LIVER GLYCOGENOSES Amylo-1,6-glucosidase (3.2.1.33) Glucanotransferase (2.4.1.25) GSD Type III Cori Disease MIM 232400 Glucose-6-phosphatase (3.1.3.9) GSD Type I Von Gierke Disease MIM 232200 MONO- & DI-SACCHARIDES Sucrase-isomaltase (3.2.1.10) Sucrose-Isomaltose Malabsorption MIM 609845 Lactase (3.2.1.23) (3.2.1.108) Lactose Intolerance MIM 223000 Galactokinase (2.7.1.6) Galactose Kinase Deficiency MIM 230200 Galactose-1-phosphate uridyl transferase (2.7.7.12) Classical Galactosaemia MIM 606999 PEROXISOMAL DEFECTS Alanine-glyoxylate aminotransferase (2.6.1.44) Hyperoxaluria Type 1 MIM 259900 LIPID METABOLISM Hydroxymethylglutaryl CoA lyase (4.1.3.4) Ketone Synthesis Defect MIM 246450 Medium chain acyl CoA dehydrogenase (1.3.99.3) MCAD Deficiency MIM 607008 SPHINGOLIPIDOSES Acid-β -glucosidase (3.2.1.45) Gaucher Disease MIM 230800 Hexosaminidase A (3.2.1.52) Tay Sachs Disease MIM 272800 Sphingomyelinase (3.1.4.12) Niemann-Pick Disease MIM 257200 RESPIRATORY CHAIN DEFECTS Cytochrome c oxidase (1.9.3.1) Complex IV MIM 220110 PORPHYRIAS Porphobilinogen deaminase (2.5.1.61) Acute Intermittent Porphyria MIM 176000 Uroporphyrinogen decarboxylase (4.1.1.37) Porphyria Cutanea Tarda MIM 176100 Protoporphyrinogen oxidase (1.3.3.4) Variegate Porphyria MIM 176200 © 2006 International Union of Biochemistry and Molecular Biology www.iubmb.org Designed by Donald Nicholson, The University of Leeds, England – and Sigma-Aldrich in collaboration with Mick Henderson, St.James’ Hospital, Leeds – and the UK MetBioNet Training Group www.metbio.net. For further information about metabolic disorders visit the OMIM (Online Mendelian Inheritance in Man) web site, hosted by the John Hopkins University – www.ncbi.nlm.gov/entrez/query/fcgi?db=OMIM GDS sigma-aldrich.com/pathways 2.3.3.10
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Transcript of Inborn Errors of Metabolism - Sigma-Aldrich -CO-OCH 1.1.1.30 CH 33COCH CH32COCH COO CH 32CH(OH)CH...
2H+
H+
OXALOACETATE
PYRUVATE
SUCCINYL-CoA
GLUTARATE
CITRATE
MALATE
2-OXO- ASPARTATE
NO2-
NO3-
N2
NH4
CH3COSCoA
ACETYL-CoA
HOH
CO2
NAD
TRA
NSAMINATION
2H+
2H+
+
CO2
5-Amino-levulinate
Glycine
H+H+
H+H+
H+H+
c
H+ H+
F0
F1
F1
β2
1 αβ
α
γ
c-sub-units
AD
P+ Pi
3.6.1.34
H
+-transporting ATP synthase
α
δF6
oscp
ATP
ADP + Pi
FUMARATE
UQH2
UQ
UQH2
NADH+H +CH2COO-
C(OH)COO-
CH2COO-
CH(OH)COO-
CHCOO-
2COO-
-OOCCOCH2CH2COO-
-OOCCH2CH2CO.SCoA
-OOCCOCH2COO-
CH3CH(OH)CH2CO.SCoA
-OOCCH=CHCOO-
Glyoxylate Cycle
-OOCCHO
GTP
GDPATP
+
HEME Protoporphyrinogen Coproporphyrinogen Uroporphyrinogen Porphobilinogen
5-Amino-levulinate
COO-
CH2
CH2H2NCH2C=O
FADH2
FADCyt.b
Fe-S
SUCCINATEII
CH3COCOO-
PYRUVATE
Pi
X Y
Cyt.c1
Fe-S
2e-
2e-1e-
1e-
III
2UQ UQ
UQH22UQH2
2UQ._
UQ._
Cyt.bL Cyt.bH
Cyt.c
IV
CuA
CuB
CuA
2e-
Heme a
Heme a3
2H+
2H+4H+
4H+
2H+
NAD+
NADH+H+
NAD+
1.1.1.39
1.2.4.12.3.1.123.1.3.43
4.1.1.32
2.3.3.1
4.2.1.3
1.1.1.41
1.2.4.22.3.1.61
6.2.1.4-OOCCH2CH2COO-
1.3.5.1
4.2.1.2
1.1.1.37
6.4.1.1
2.3.3.8
4.1.3.1
2.3.3.9
ISOCITRATE
2.6.1.12.3.1.16
4.1.1.71
1.2.1.16
4.3.1.1
1.4.1.2 1.4.1.14
6.3.5.4
1.7.1.11.7.99.4 1.7.7.1
1.7.1.4
1.18.6.1
2.3.1.37
1.10.2.2
1.10.2.2
γ
εδ
β
33 β
ATP
ADP
ENDERGONIC REACTION
1.6.5.3
I
FMNH2
FMN I
1.6. 5.3
2Fe -S (5 Clusters)
H+ H+H+ H+
ATP
4H+
2H+
2H+or
H+
PHOTO-SYSTEM
l
O2
3.6.3.14
NADP+
GlyceraldehydeRibulose-1,5-bis-P
2β
88
H+c
32
ε
a
32
α1
α
ε
α
β2
1
3
α
αβ
3
α
H+
H+
H+
H+
H+H+
H+
H+ H+H+
H+H+H+H+
H+
H+H+H+
H+H+
H+
H+
H+
H+
H+
H+H+
H+H+
H+H+
H+H+H+
Translocated protons
H+
H+H+
H+Pi
Ferredoxin
4H+PC PC
PC
PQH2
2PQH2
PQ_.
2PQ_.Fe-S Cyt.f
PQ
2PQ
Cyt bf
2e-
1e- 1e-
2e-
2e-
2e-
2e-
Cyclic Photophosphorylation
PQ
PHOTO-SYSTEM II
Cyt bc
Non-cyclic electron flow
2e-2e-
Mn2e-
*
Protons from Water
H2O
H+
H+
2H+
2H+
γCO2
Fixation
H+
ADPPi
H+
NADPH+H+
ATPTHYLAKOID MEMBRANE
CHLOROPLAST OUTER MEMBRANE
THYLAKOID LUMEN
STROMA
(electric current)
P680Chl.a
QA
QB
Pheophytin
P700
Chl.A0
A1
Fe-S
ATP
ADP
ATP
1/2O2
H2O
PQH2
To Brain -
AMINO
ACIDS
PYRI
MIDINES
PURINES
CATECHOLAMINES
AROMATIC
AMINO
ACIDS
hvVISION
LIPID
PHOTOSYNTHESIS
PORPHYRINS
STEROIDS
ISOPRENOIDS
PHOSPHOLIPIDS
DEGRADATION
LIPID
BIOSYNTHESIS
PENTOSES
HEXOSES
POLYSACCHARIDES
COCH CH(NH )COO2 3+
FOLICACID
C1POOL
VALINE
ATP
ATP
4.1.1.4 1.1.1.30
CH COCH3 3
CH COCH COO3 2
CH CH(OH)CH COO3 2
3-OH-ButyrateAcetone
Acetoacetate
KETONE BODIES
HYALURONIC ACID DERMATAN BLOOD GROUPSUBSTANCES
PEPTIDO-GLYCAN
N-Ac-Neuraminate(Sialate)
UDP-Iduronate
UDP-N-Ac-Galactosamine UDP-
Galacturonate
GDP-Fucose
TDP-Rhamnose
ADP-Glucose
UDP-Glucose
TDP-4-Oxo-6-deoxyglucose
MANNOSE
CHITIN CHONDROITIN PECTIN
ALGINATES
INULIN CELLULOSE
O-ANTIGENS STARCH GLYCOGEN
LACTOSE
GALACTOSE
O
OH
HO
OH
OH
CH3 O
OPPTO
OH
OH
O
OHACNH
HO OH
O
OHHO OPPU
NHAC
OH
OH OH
OH
HO
OH
NHCOCH
OH HO
O
OPPG
CH OH2
HO
P
UDP-N-Ac-Glucosamine
pyruvateN-Ac-Mannosamine-6-P UDP-Glucuronate
GDP-Mannose
N-Ac-Mannosamine UDP-N-Ac-Glucosamine N-Ac-Glucosamine-6-P
Sorbitol
Fructose-1-P
Erythrose-4-P
3-Dehydrogulonate
L-Xylose
L-Lyxose
D-Xylulose
D-Ribose
RibitolL-Arabitol L-Xylulose
L-Ribulose L-Ribulose-5-P
L-Xylulose-5-P
L-Arabinose Xylitol D-Xylose
D-Arabinose D-Ribulose
ASCORBATE
2, 3-Dioxogulonate
N-Ac-Glucosamine-1-P
Inositol-PInositolGlucuronateGulonate
Gulonolactone 2-Oxogulonolactone
Fructose
6-P-Gluconate
D-Ribulose-5-P
D-Xylulose-5-P
Mannose-1-P
GDP-Glucose
TDP-Glucose
UDP-Galactose
Galactose-P
Glucose-1-P
Glucose-6-P
Fructose-6-P
Fructose-1:6-bis-P
OH
H H HO
HOCH2 C C C C CO
OH OHH OH
H HO
HOCH2 C C C CO CO
OHH
H
OH H
C C
OH
CO
P-Ribosylamine
Glycerate2, 3-Diphospho-glycerate
3-P-Glyceraldehyde
1:3-bis-P-Glycerate
3-P-Glycerate
2-P-Glycerate
P-enolpyruvate
Inositol
CH OH2
CH OH2
HOCH
CH OH2
CH O2
HOCHP
Glycerol
UDP-GalactoseUDP-Sugars
CH CH OH3 2
ETHANOL
Dihydroxy-acetone-P
(Glycerone-P)
Glucosamine-6-P
Phospho-serine
Chain elongation Mitochondrial
Serine
Serine
Indole-3-glycerol-P
3-Deoxy-D-arabino-heptulosonate-7-P
Indoleacetate(Auxin)
Indoxyl
Formylkynurenine Kynurenine 3-Hydroxykynurenine
3-Hydroxyanthranilate
2-Amino-3-carboxymuconate semialdehyde
NH
OH
Tryptamine 1-(o-Carboxy phenylamino)1-deoxyribulose-5-P
N-(5-P-Ribosyl)anthranilate
Anthranilate
Catechol
NH
Indole-acetaldehyde
NH
CH CHO2
Indolepyruvate
NH
C-CH(OH)CH(OH)CH O2 PCH
2-Aminomuconate-6-semialdehyde
CH CH NH2 2 2
NH
1.2.3.7
4.1.1.43
3.5.1.9 3.7.1.3
4.2.1.20
1.13.11.6 4.1.1.45
2.4.2.18
4.2.3.5
4.1.1.48
1.14.13.9
4.2.3.4 Dehydro-quinate
4.2.1.10 1.1.1.25 2.5.1.19Shikimate-3-P Shikimate-5enolpyruvate 3-P
ChorismatePEP
2.7.1.71
1.4.1.19
Homogentisate Phenylpyruvate
Cinnamate
Coumarate
Prephenate
2-Aminomuconate
Fumarate Aspartate
GLYCINE
Sarcosine
Hydroxy-pyruvate
P-Hydroxy-pyruvate
SERINE
Imidazoleglycerol-P
P-Ribosyl-ATP P-Ribosylformimino5-aminoimidazole-carboxamide-R P
Formiminoglutamate
Imidazolonepropionate
Urocanate
Cystathionine Homocysteine
Phosphoadenylyl-sulphate
(PAPS)
CH (NH )COOH2 3+
Histidinol-PHistidinolHistidinal3.1.3.15 2.6.1.91.1.1.23
3.6.1.31
5.3.1.162.4.2.17
Asparagine
ALANINE
3-Sulphinylpyruvate
Oxobutyrate
2-Aceto-2-hydroxy-butyrate
O-Phospho-homoserine
HO SCH CH NH2 2 2 2
HypotaurineHO SCH CH NH3 2 2 2
Taurine
Glutamate
γ-Glutamylcysteine
Glutathione
Glycine
Cysteine
β-Alanine
Cysteate S-Adenosylhomocysteine
4.2.3.1
1.1.1.864.2.1.9
1.1.1.86 4.2.1.9 2.6.1.32
4.1.3.18
S-Adenosylmethionine
(SAM)
2-Methylaceto-acetyl-CoA
2-Acetolactate 2-3-Dihydroxyisovalerate
2-Oxo-isovalerate
3-Hydroxy-isobutyrate
3-Hydroxy-Isobutyryl-CoA
Methylacrylyl-CoA
2-Methyl-3-hydroxy-
butyryl-CoA
Tiglyl-CoA 2 Methylbutyryl-CoA
2:3-Di-OH-3-methylvalerate
2-Oxo-3-methylvalerate
Isobutyryl-CoA
Cysteinesulphinate
2.5.1.48
2-Isopropyl-malate
CH CH COCOO3 2
+CH CH(NH )COO3 3
CH CH(OH)COO3
HO SCH COCOO2 2
OCH COCOO2P
OCH CH(NH )COO2 3P
HOCH COCOO2
HOCH CH(NH )COO2 3+
+
OH
CH COCOO2
COO
OHOHO
COO
OHOH
HO P
COO
OHOH
O
CH2
P
COO
O-C-COOOH
OOH
OOC CH COCOO2
N H
HOC-CH(OH)CH(OH)CH 2OPCH
COO
NH2
COO
OHNH2COO
COO
OCH
OOCCH NHCH2 3
CH (CH ) COS-ACP3 2 14 CH (CH ) COSCoA3 2 14
CH (CH ) CH=CHCO-S-ACP3 2 2
CH CH=CHCO-S-ACP3 CH CH(OH)CH COS-ACP3 2
COSCoA
Linoleate
Oleoyl-CoA
Stearoyl-CoA Dehydrostearoyl-CoA OH-Stearoyl-CoA Oxostearoyl-CoA
Palmitoyl-ACP Palmitoyl-CoA
ACYL-ACP 2, 3-Enoyl-ACP
3, 4-Decenoyl-ACP
2, 3-Decenoyl-ACP
3-OH-Acyl-ACP 3-Oxoacyl-ACP
3-Oxo-Decanoyl-ACP
3-Oxo-Hexanoyl-ACP3-OH-Hexanoyl-ACP2, 3-Hexenoyl-ACP
Butanoyl-ACP Crotonoyl-ACP 3-OH-Butanoyl-ACP Acetoacetyl-ACP
Hexanoyl-ACP
3-OH-Decanoyl-ACPDecanoyl-ACP
Palmitoleoyl-ACP
γ-Linolenate Arachidonate Leukotriene B4
COSCoA
CO-S-ACP
Thromboxane B2
HOOCCH CO-S-ACP2
Malonyl-ACP
HOOCCH CO-SCoA2
Malonyl-Co-A
CH CO-S-ACP3
Acetyl-ACP
CH O-CO-R2
CH OH2
R’-CO-OCH
FATTY ACIDACYL-CoA(Cytosol)
CH O-CO-R2
CH O2
R’-CO-OCH
P
Cardiolipin Phosphatidylglycerol
AcetylcholineGlycerophosphocholine
Lysolecithin
Cholineplasmalogen
CDP-choline Choline-P
Mevaldate
CH (CH ) CH=CHCH(OH)CHCH OH3 2 12 2
Dehydrosphingosine Sphingosine 4-Sphingenin Psychosine
Acyl-CoAAcyl-CoA
CerebrosideGalactoseCH (CH ) CH=CHCH(OH)CHCH O-3 2 12 2
NHCOR
Isopentenyl-PP(C5)
Dimethylallyl-PP(C5)
Geranyl-geranyl-PP(C20)
Farnesyl-PP(C15)
Squalene(C30)
Geranyl-PP(C10)
Desmosterol Zymosterol Lanosterol
CH C = CHCH O3 2 PP
CH3
Ceramide
Gangliosides
NHCOR
CH O2 PP
OPPCH2
CHOLESTEROL
CH C(OH)CH CH OH3 2 2
CH COO2
CH C(OH)CH CHO3 2
CH COO2
NADP+Dehydroascorbate
OH
H O
HOCH2 C C C C CO
OHH OH
OH
H O
HOCH2 C C CO CO CO
H
NAD+Pi
ADPADP
Phytoene(C40)
Lycopene (C40)
β-CAROTENE (C40)RhodopsinMetarhodopsin
Retinoate
trans-Retinal
11-cis-Retinoltrans-Retinol(Vitamin A)
Retinol esters
Dark
Light
Ubiquinone(Coenzyme Q)
Menaquinone
Plastoquinone
Phylloquinone(Vitamin K)
Opsin
CHO
CH OH2
CHO
CH OH2
CH3CH O3
CH O3 n
O
O
O
O
Phytol (C20)
α-Tocopherol(Vitamin E)
Quinolinate
Quinolinate-nucleotide
Nicotinate-nucleotide
Desamino-NAD
5-Hydroxy-tryptophan
5-Hydroxytryptamine(SEROTONIN)
N-Acetyl-5-O-methyl-serotonin(MELATONIN)
NH
CH CH NHCOCH2 2 3HO
NH
CH CH NHCOCH2 2 3CH O3
NAD(P)+
NICOTINATE
N-Acetyl-serotonin
2.4.2.19
Dopamine Dopa
Dopaquinone
THYROXINEMELANIN
OHOH
CHOHCH NHCH2 3
OHOH
CHOHCH NH2 2
4-OH-3-Methoxy-phenylglycol
CHOHCH NH2 2
OHOCH3OCH3
CHOHCH OH2
OH
OCH3
4-OH-3-Methoxy-D-mandelate
Cyclic AMP2H-folate
4H-folate
ATP
Dephospho-Coenzyme A
4-P-Pantetheine
4-P-Pantothenylcysteine
4-P-Pantothenate
Pantoate
6.3.2.1
1.1.1.169
3.5.1.22
2.7.1.33
NH
NH
O
O
O
O
CH OH2
OHOCH3
CH(OH)COO
H2
NH3+
CH-COOCO
O
COO
NRibose- PN+
COO
NH
CH COO2
O
OH
CH O2 P
NHCOCH NH2 2
OHFormyl
glycinamide-R P
Urea
Formylglycinamidine-R P
Allantoate Allantoin URATE
ADP
Xanthine Hypoxanthine
Inosine
Adenosine
Adenylo-succinate
5-Aminoimidazole-R P
5-Amino-4-imidazolecarboxylate-R P
5-Amino-4-imidazole(N-succinylcarboxamide)-R P
5-Aminoimidazolecarboxamide-R P
Formylamido-imidazole-
carboxamide-R P
H NCONH2 2
HNH C2 CHO
NHRP
NH
C
CCO
CCO
OCNH
NH
HN
HN
H
CCO
CCH
OCNH
N
N
HN
Carbamoylβ-alanine
Dihydro-uracil Uracil
d-ADP
d-ATP
GTP GDP
XANTHOSINE-P(XMP)
TTP
TDP
β-Ureidoisobutyrate
Dihydrothymine
Thymine d-UMP d-CMP d-CDP
CDP
Carbamoylaspartate
Dihydroorotate
Orotate Orotidine-P Uridine-P(UMP)
UDP
3-Amino-isobutyrate
CHCNH2
CHOCNH
NCHCO
CHOCN H
HN
CH-CH3CO
OCNH
HNCH2
CH2CH2
CO
OCNH
HN
CO
CHOCNH
HN C CH3
Methylmalonylsemialdehyde
OHCCHCOOCH3
CH2CO
CH-COOOCNH
HN CHCO
C-COOOCNH
HN
H NCONHCH CH COO2 2 2
CC
CCH
RPHC
NH
NH
OOC-CH-CH COO2
N
N
N
CHO
NHRP
NH
OC
H C2
2.7.7.433.1.3.29
1.1.1.158
5.1.3.13
3.1.3.294.1.3.20
2.7.1.60
5.1.3.14
5.1.3.7
5.1.3.1
2.7.1.4
5.4.2.3
3.1.1.17
1.1.1.49
1.1.1.21
2.7.7.23
3.1.3.251.13.99.11.1.1.19
1.1.3.8
5.3.1.3
2.7.1.53
1.1.1.92.7.1.15
2.7.1.17
5.1.3.4
5.1.3.1
2.2.1.2
4.1.2.13
2.7.1.11
2.7.1.47
2.2.1.1
1.2.1.12
1.1.1.29
1.1.1.95
1.3.1.35
1.14.19.3
2.3.1.41
2.3.1.41
2.3.1.41
1.1.1.100
5.3.99.5
4.2.1.60
5.3.99.31.14.99.1
1.13.11.34
1.3.1.101.3.1.9
1.14.19.1
1.3.1.9
4.2.1.61
4.2.1.60
4.2.1.60
4.2.1.59
4.2.1.58
1.3.1.9
1.3.1.9
6.2.1.3 3.1.2.20
1.1.1.100
5.3.1.1
HOOC-COOHOxalate
Glycolate
HOCH CHO2
Glycolaldehyde
Ethanolamine-P
2.7.1.30
2.3.1.7
3.7.1.2
2.7.8.8
2.1.1.172.1.1.71
1.3.1.35 2.7.8.2
3.1.4.3
3.1.4.4
2.7.7.15
3.1.1.5 3.1.4.2
2.7.1.32
2.7.1.82
1.2.4.12.3.1.121.8.1.4
2.6.1.21.4.1.1
4.1.1.1
2.3.1.50
1.1.1.102
2.7.8.3
2.4.1.62
3.2.1.46
5.2.1.31.2.1.36
2.3.1.763.1.1.21
5.2.1.7
5.4.99.71.14.99.7
4.2.1.75
1.3.3.3
4.99.1.1
2.5.1.29
4.1.1.28 2.3.1.5 2.1.1.4
6.3.5.16.3.1.5
2.4.2.11
2.7.7.18
2.6.1.5
1.2.1.32
1.13.11.5
2.1.1.28
2.1.2.2 6.3.3.1
3.5.2.5
1.4.1.10
1.7.3.3 1.1.3.22
4.1.1.28
4.1.1.21
2.1.2.3
2.4.2.1
1.5.99.2
2.1.1.5
2.6.1.22
3.5.2.3
2.6.1.511.4.1.7
3.1.3.3
2.6.1.52
2.1.3.2
ACETATE
Glycine
2.4.2.4
2.1.1.45
RP
CHCO
CHOC N
HN
1.3.1.14 2.4.2.10 4.1.1.23
2.7.7.7
2.7.7.7
2.7.7.7
1.17.4.1
2.7.7.7
2.7.4.14
4.4.1.1
1.8.1.6
1.13.11.20
4.4.1.8
4.2.1.22
1.1.1.27
4.1.1.29 6.3.2.3
6.3.2.2
1.8.1.3
LACTATE
Acetaldehyde
2.7.1.24
2.7.7.3
4.1.1.36
Bile Acids
CH CH COSCoA3 2Propanoyl-CoA
1.1.1.31
2.1.3.14.1.1.415.1.99.1
6.3.2.5
2.1.1.142.1.1.13
4.6.1.1
CDP-Ethanolamine
1.1.1.35 4.2.1.17
+HO SCH CH(NH )COO2 2 3
3.5.2.7NHHN
CH
OOC CHCH CH COO2 2
CMP-N-Acetylneuraminate
CDP-diacylglycerol
CHOLINE
Ethanolamine
Glyoxylate
HISTAMINE
2.3.1.46Homoserine
Tyramine
Plant PigmentsTannins
Maleylacetoacetate
Fumarylacetoacetate
5.2.1.2
CH COO2
OH
OH
OH
CH CH NH2 2 2
OHOH
CH CH NH2 2 2
Hydroxyphenylpyruvate
α-Tocopherol(Vitamin E)
LIGNIN
d-GTP
OH
OH OH
O
HO
OH
P
OPPT
CH3HO
OHOH
O
NADPH
2.2.1.1
2.7.1.3
1.1.1.45
1.1.1.130
1.10.3.31.10.2.1
1.1.1.10
ATP
CH CH NH2 2 2
NH
HO
5.4.99.5
2.6.1.51.13.11.27
Indole
4.1.99.1
1.13.11.11
OHO
OH
NH C C CH CH O2 P
H HCOO
NH2
COO
6.3.5.3 6.3.2.6 4.3.2.2
GuanineDNA
6.3.4.44.3.2.2
CCO
CCH
HCNH
N
N
HN CCO
CCH
HCNH
N
N
HN
3.5.3.4
2.4.2.1
2.7.7.6 2.7.7.6
2.7.7.6
2.7.7.6
2.7.4.62.7.4.32.7.4.4
2.7.4.6
2.4.2.15
1.1.1.205
6.3.4.16.3.5.2
2.7.4.4
3.5.4.3
3.2.2.2
2.4.2.1
3.1.3.5
3.1.22.1
6.3.4.2
1.17.4.1
1.17.4.1
3.5.4.12
2.7.4.8
2.7.4.9
2.7.4.6
2.4.2.4
1.3.1.2
3.5.2.23.5.1.6
3.5.1.6 3.5.2.2 3.5.4.1
Diphospho-mevalonate
Mevalonate
3-Oxopentanoyl-CoA
3-Oxoacyl-CoA3-OH-Acyl-CoA2, 3-Enoyl-CoA
2, 3-Hexenoyl-CoA 3-OH-Hexanoyl-CoA
Acetoacetyl-CoA3-OH-Butanoyl-CoACrotonoyl-CoA
Pentanoyl-CoA 3-OH-Pentanoyl-CoA
1.1.1.35
1.1.1.35
1.1.1.157
1.3.99.3
1.3.99.2
1.1.1.35
Odd C Fatty acids
Succinylhomoserine
CO2
2-OXO ACID
Glutamyl-P
Glutamine
UREA
P-CreatineCreatine Creatinine
Glycine
CITRULLINE
Glutamic semialdehyde
2-AMINO ACID
Pyrroline-5-carboxylate
GlyoxylatePyruvate
4-Hydroxy-2-oxoglutarate
4-Hydroxy-glutamate
3-Hydroxy-pyrroline-
5-carboxylate
PutrescineH NCH CH CH CH NH2 2 2 2 2 2
Spermidine
Spermine
N -Trimethyllysine66N -Trimethyl-3-OH-lysine
Carnitine
Glutaryl-CoA Saccharopine2-Aminoadipatesemialdehyde
2-Aminoadipate2-Oxoadipate
Arginino-succinate
Guanidoacetate
Methylmalonyl-CoA
4.1.2.12
AspartylSemialdehyde
2, 3-Dihydro-dipicolinate
1.2.1.11
4.2.1.52 1.3.1.26 Piperideine-2, 6-dicarboxylate
N-Succinyl-2-amino-6-oxo-
pimelate
2.6.1.17N-Succinyl-2, 6diaminopimelate
Diamino-pimelate
3.5.1.18
3-Methyl-glutaconyl-CoA
(CH ) CHCH COSCoA3 2 2(CH ) CHCH COSCoA3 2 2
3-Methyl-crotonyl-CoA
Isovaleryl-CoA
OxopantoateHCHO
OOCCH C = CHCOSCoA2
CH3
CH COCHCOSCoA3
CH3
CH CH=CHCOSCoA3
CH3CH CH(OH)CHCOSCoA3
CH3
C(OH)CH(OH)COO
CH CH3 2
CH3
CH = CCOSCoA2
CH3
HOCH CHCOS-CoA-2
CH3
CH CHCO-SCoA3
CH3
C (OH)CH(OH)COOCH3
CH3
CH CH CHCOSCoA3 2
CH3
CH C = CHCOSCoA3
CH3
ATP
4-Aminobutyrate(GABA)
1.3.99.7
1.5.1.2
1.14.11.2
2.6.1.39 1.2.1.31
1.14.11.8
2.5.1.16
2.5.1.22
4.1.3.16
2.6.1.23
1.5.99.8
1.5.1.2
6.4.1.4
4.2.1.33
2.6.1.6
1.1.1.85Oxoleucine
COOH
(CH ) CHCHCH(OH)COO3 2
S-Adenosylmethylthiopropylamine
(Decarboxylated SAM)
PROLINE
Coenzyme A
CH C= CHCH3 2
CH3
OCH
CH
3
3HO
CH3CH3
CH3
CH C-CH H O3 2 2C PP
CH2
CH C= CHCH O2 2 PP
CH3
3-P-Glycerol
Phosphatidylethanolamine
CEPHALIN
3.1.4.12
Progesterone
1.5.1.12
2.7.3.2
4.2.1.173.1.2.4
2.6.1.32
4.4.1.15
Acetylserine 2.7.7.42.7.1.251.8.99.1 Adenylylsulphate
(APS)
2.7.1.39
4.4.1.15
4.1.1.29
2.5.1.47
2.3.1.30
P-Ribosyl-PP
1.1.1.1
1.2.1.4
Aspartyl-P
5.1.1.7
2.7.1.40
2.2.1.6
4.1.3.18
1.5.1.9
1.14.11.1
CHOLINE
2.1.1.13
1.3.1.2
NHCH CH SH2 2 NHCH CH CO2 2OCH C(CH ) CH(OH)CO2 3 2P
ADP- NHCH CH SH2 2 NHCH CH CO2 2OCH C(CH ) CH(OH)CO2 3 2
P-ADP- NHCH CH SH2 2 NHCH CH CO2 2OCH C(CH ) CH(OH)CO2 3 2
1.4.1.8
1.3.99.3
1.3.99.3
1.4.1.9
4.2.1.18
4.1.1.20
1.3.99.7
1.8.99.2
+CH CO-OCH CH(NH )COO2 2 3
2.5.1.6
2.1.1.102.1.1.20
1.1.1.3
1.1.1.3
4.2.1.18
4.1.2.5
4.3.1.5
4.2.1.20
2.1.
1.20
Glyoxylate
2.1.1.6
1.4.3.4
Hexanoyl-CoA
Butanoyl-CoA
6.3.5.5
2.7.2.11
NO1.14.13.39
3.5.3.63.5.3.1
4.1.1.17
2.7.7.41
2.6.1.4
1.1.1.100
1.1.1.8
2.3.1.512.3.1.15
2.7.8.5
SO -42
1.14.16.4
NH2 OOC OOC
4.1.1.25
1.3.1.13
1.3.1.13
2.7.4.6
CYTIDINE-triphosphate
(CTP)
Cytosine
2.4.2.9
4.1.1.11
2.6.1.18
4.3.1.3
4.2.1.49
3HSO-
1.1.1.105
2.3.1.41
NADPH
1.1.1.22
2.3.1.4
5.5.1.4
3.2.1.26 3.2.1.48
2.6.1.16
1.1.1.14
2.7.7.34
4.2.1.47
2.4.1.682.4.1.69
2.4.1.9
5.3.1.8
2.4.1.11
O
OPPU
CH OH2
CH OH2
HO OH
OH
HO
2.4.1.21
4.2.1.52
1.2.1.18
4.2.1.18
O
CHC
CHOCN
HN
DP
CHCO
CHOCN
HN
RPPP
OCH2
H
C C
H
OHOH
CH
NHNCH
CP
H
O
OCH2 C C
H
OH
H
OH
C
H
C CCNH2
CCH
HCN
RP(PP)
N
N
N+
P
NH2H
O
OCH2 C C
H
OH
H
OH
C
H
C COC
CCH
HCN
RP
N
N
NHP
H
OCH2 C C
H
OHOH
CO CH2 C
CONH2
CCH
HCN
RP
N
N
NHP
+OOCCH N(CH )2 3 3
Betaine
Betainealdehyde
2.4.1.16
5.1.3.6
2.4.1.33
2.7.7.13
5.4.2.8
5.3.1.8
O
OH
CH OH2
HO
O PHO
2.7.1.28
2.7.1.31
6.3.4.13
5.3.1.1
2.2.1.1
4.1.1.9
3.1.2.11
β-OH-β-Methyl-glutaryl-CoA
6.3.4.5
5.3.1.6
2.5.1.21
2.1.1.2
3.5.2.10
6.3.2.136.3.2.7-10
2.7.7.27
2.7.7.9
3.5.4.10
CCO
CCH
OCN RP
N
NH
HN
CCO
CCH
N
N
N
HN
RPH N2 C
2.7.4.6
1.17.4.1
2.7.4.6
2.6.1.13
Carbamoyl-P
2.3.1.9
Carnitine
2.6.1.4 2.6.1.44
CH C(OH)CH COSCoA3 2
CH COO2
1.2.3.5
1.1.1.34
2.3.1.16
4.1.3.5
2.3.1.16
Menaquinone
4.1.1.15
4.3.1.19
Carnosine
CH CH NH2 2 2C
NHNC
HC
H
4.1.1.22
4.3.1.3
Ubiquinone
Plastoquinone
D-Ribose-5-P
NH
CH CH(NH )COO2 3HO+
O
OH
OH
HO
OPPU
COO-
O
OH
OH
HO OPPU
COO-
OH
H OH
HOCH2 C C CO
H
CO COO-
OCHOHCHOHAcNH
HO
OPC
OCO -CH OH2
OCHOHCHOHAcNH
HO
OH
COOCH OH2
O
OHOHHOHO
CH O2 P
O
OHACNH
HO OH
CH O2 P
O
OOHHOHO P
CH OH2
O
OHOH HOHO
CH OH2
O
OHHO OH
3
CH O2 PO
OHHO O
NHCOCH3
P
CH OH2CH OH2CH OH2
O
OHHO OH
NH2
CH O2 P
O
OH
HO
OH
OPPU
CH OH2
O
O
HO
OH
OH
HO
P
CH OH2
CH OH2O
OHO
OH
OH
O
OHOH
OH
CH OH2
O
OHHO
OH
OH
CH2CH OP2
OH
H H HOH
C C C C
OH OHH
COO-HOCH2
H
OH OHOH
C C C CHO
HH
HOCH2
H
OH OHH
C C C CHO
HOH
HOCH2
OH
H OHH
C C C CHO
HOH
HOCH2
H
OH HOH
C C C CHO
OHH
HOCH2
OH
H HOH
C C C CHO
OHH
HOCH2
OH
H OHOH
C C C CHO
HH
HOCH2
H
OH HOH
C C C C
OHH H
OH
COO-POCH2
OH
H OH
C C CO
H
POCH2 CH OH2
H
OH H
C C CO
OH
HOCH2 CH OH2
CH OH2POCH2
H
OH OH
C C CO
H
CH OH2POCH2
OH
H H
C C CO
OH
CH OH2POCH2
H
OH OH
C C CO
H
HOCH2 CH OH2
OH
H OH
C C CO
H
HOCH2 CH OH2
OH
H H
C C CO
OH
HOCH2 CH OH2
H
OH OHH
C C C
HOH
HOCH2 CH OH2
OH
H OHOH
C C C
HH
HOCH2 CH OH2
H
OH HOH
C C C C
OHH H
OH
CH OH2HOCH2
H
OH OH
C C CHO
H
POCH2
H
OH HOH
C C C CO
OHH
HOCH2 CH O2 P
H
OH OHOH
C C C
HH
HOCH2 CH OH2
H
OH OH
C C C
H
CO
H
HO
POCH2 CH OH2
P
H
OH OH
C C C
H
CO
H
HO
CH O2 POCH2
H
OH OH
C C
H
C CHO
OH
H
POCH2POCH2
O
OH
NH2
OH
CH O2 P
CHOHCHOPOCH2
HOCH2COCH2OP
O
OH
O OP P
OH
CH O2 P
CHOHCOOPPOCH2
CHOHPOCH2 COO
COO
OH
OHOOH
COO
CH (CH ) CH(OH)CH COS-CoA3 2 14 2CH (CH ) CH(OH)CH COS3 2 14 2
CH (CH ) COCH COS-CoA3 2 14 2
CH (CH ) COCH COS-CoA3 2 14 2
CH (CH ) COS-CoA3 2 n+2
CH (CH ) CH=CHCOS-CoA3 2 14
CH (CH )3 2 nCH=CHCOS-CoA
CH (CH )3 2 5 2CH=CHCH COSACP
CH (CH )3 2 6CH=CHCOSACP
CH (CH )3 2 6COCH COSACP2CH (CH )3 2 6 2 2CH CH COSACP
CH (CH )3 2 2 2 2CH CH COSACP
CH CH CH COSACP3 2 2
CH (CH )3 2 6CH(OH)CH COSACP2
CH CH=CHCO.S-ACP3
CH (CH )3 2 2 2COCH COSACP
CH3COCH COSACP2
CH (CH )3 2 n 2COCH COSACPCH (CH )3 2 n 2CH(OH)CH COSACP
CH (CH )3 2 2 2CH(OH)CH COSACP
ACYL-CoA(Mitochondria)
CH (CH ) CH CH COSCoA3 2 2 2n
CH (CH ) CH=CHCOSCoA3 2 n CH (CH CH(OH)CH COSCoA3 2n
CH (CH ) CH CH COSCoA3 2 2 2 2 CH (CH ) CH=CHCOSCoA3 2 2
CH (CH ) COCH COSCoA3 2 2n
CH COCH COSCoA3 2
CH (CH ) CH(OH)CH COSCoA3 2 2 2
CH CH(OH)CH COSCoA3 2CH CH=CHCOSCoA3CH CH CH COSCoA3 2 2
CH CH CH CH COSCoA3 2 2 2
Pentenoyl-CoACH CH CH=CHCOSCoA3 2 CH CH CH(OH)CH COSCoA3 2 2
CH (CH ) COCH COSCoA3 2 2 2
CH CH COCH COSCoA3 2 2
OH OH
HO OH
O
O-CH O-PO2
CH CHOHCH OH2 2
O
CH2
CH2
R'-CO-OCH
O-CO-R
O-PO
O-
HOCH CH2 2NH3+POCH CH2 2NH3
+CPP- OCH CH2 2NH3
+
O
-
CH O-CO-R2
CH O P2 OCH CH2 2NH3+
CH COCH CH N(CH )3 2 2 3 3
HOCH CH N(CH )2 2 3 3
+
CH CH 2 COO(NH )3+
OHOH
CH CH 2 COO- (NH )3+
CO
NH2
CH CH(NH )COO2 3+
NHOC
CO
NHOC
NH
CH
H N2
HCCH
N
N
C
RPH N2
CHC
CHOCN
N
DP
NH2
RPPP
CHC
CHOCN
N
NH2
CC
CCH
RP(P)HC
N
N
N
N
NH2
H NCOO2 P
(CH ) NH2 3 2H N(CH ) NH2 2 3 (CH ) NH2 4
(CH ) NH2 3 2H N(CH ) NH2 2 4
CH -SCH CH CHNH3 2 2 2
Adenosyl
+
+CH3
CH3
CHCH(NH )COO3
++C
NHNC
HC
H
CH CH(NH )CH OH2 3 2
+C
NHNC
HC
H
CH CH(NH )CH OP2 3 2C
NHNCH
HC CH COCH OP2 2
+HSCH CH CH(NH )COO2 2 3
+
+SCH CH CH(NH )COO2 2 3
CH CH(NH )COO2 3
RP
CO
C-COOOCN
HN CH
2.4.1.23
CH (CH ) CH=CHCH(OH)CHCH OH3 2 12 2
NH3+
CH (CH ) CH(OH)CHCH OH3 2 14 2
NH3+
CH (CH ) COCHCH OH3 2 14 2
NH3+
GalactoseCH (CH ) CH=CHCH(OH)CHCH O-3 2 12 2
NH3+
4.1.1.70
H NCONH2 2
OHCCH N(CH )2 3 3+
C-CH3CO
CHOCN DP
HN
DP
CPP-OCH CH N(CH )2 2 3 3+
OCH CH N(CH )2 2 3 3
+
1.2.1.41
UDP-N-Ac-Muramate
O
O
CH CH3
COO-
NHAC
HO OPPU
CH OH2
2.5.1.57
O
OH
CH3
HO HO OPPG
2.7.7.24
2.4.1.22
2.7.7.12
5.1.3.2
5.3.1.4
Sedoheptulose-PP
3.1.1.28
4.2.1.24
N
CH2
CH2
N
N
N
CH3
CH3
CH
CH
COO
CHCH
CH2
H C3
H C3H C3
HC
C
C
H
H
Fe
CH2
CH2
COO- -
CH3
CH3
CH2
CH2
CH2
H C3
H C3N
H
NH
NH
NH
CH
CH
H C2
C
CH2
H2
-
CH2
CH2
COO
CH2
CH2
COO -
H C2
H C2
CH 2
CH 2
OOC
COO
H N2
NH
-
-
CH2
H C2
H C2
H C2
H2
H2
CH2
CH2 CH2
CH2
CH2
CH2
NH
NH
NH
NH
COO
COO
COOCOO
C
C
-
-
CH2
CH2
COO
CH2
CH2
COO- -
-
-
OOC-
OOC-
CH3
CH3
H C3
H C3
H C2
H2
H2
NH
NH
NH
NH
CH2
CH2
CH2
CH2
CH2
C
C
COO-
CH2
CH2
COO
CH2
CH2
COO- -
COO-
COO-
CH C(OH)CH CH O3 2 2 PP
CH COO2
OHCCOO
HOCH COO2
NH2
H NCNHCH COO2 2
+ NH2
H NCN(CH )CH COO2 3 2
+ NH2
HNCN(CH )CH COO3 2P-
+ HN CNH CO
N CH2
CH3
OOCCHCH COO2
H NCNHCH CH CH CH2 2 2 2
N(NH )3
+COO
CHCOOHC
CH2HOCH
N
OOCCH(OH)CH CH(NH )COO2 3
+
CHCOO
CH2HOCH
H C2NH
NH
CHCOO
CH2CH2
CH2
OOCCH(OH)CH COCOO2
N
CHCOO
CH2CH2
CH
CH COCOO3 OHCCOO
+H NCONHCH CH CH CH2 2 2 2 (NH )3 COO
H NOCCH CH CH2 2 2 COO(NH )3
+
R-CO-COO
+
COOR-CH(NH )3+
COOH NOCCH CH2 2 (NH3+
OOC-CH-COSCoA
CH3
P OOCCH CH2 COO(NH )3+
OOCCH CH CHO2 2
OHCCH CH2 COO(NH )3
+
(CH ) NCH CH(OH)CH COO3 3 2 2
+
OHCCH CH CH2 2
+COO(NH )3
POOCCH CH CH2 2
+COO(NH )3
OHCCH CH CH CH2 2 2 COO(NH )3+
NH CHCH CH COO2 2
COO
CH CH CH CH CH2 2 2 2 COO(NH )3
+
H N(CH )2 42 CH(NH )COO3CH(NH3++
(CH ) N(CH )3 3 2 3CH CH(NH )COO2 3+ +
CH2CH
OOCCN
HCCH-COO
CH2CH2
CH-COO OOCCN
H C2
OOCCOCH CH CH CH-COO2 2 2CH CH2 2 2
OOCCH CH CONH2 2 OOCCHCH CH CH CH-COO2 2 2
OOCCH CH CONH2 2CH2 2
NH3+
OOCCH-CH CH CH CHCOO2 2 2
NH3+
NH3+
(CH ) CHC(OH)CH3 2 2
COOH
COO (CH ) CHCH COCOO3 2 2
+(CH ) CHCH3 2 2 CH(NH )COO3
HOCH CHCOO2
CH3
CHCOCOO
CH3
CH3CH COC(OH)CH3 3
COO
+POCH CH CH(NH )COO2 2 3
+HOCH CH CH(NH )COO2 2 3
+CH CH CH(NH )COO2 2 3 OOCCH CH2COO 2
+ CH SH2
OOCCH(NH )CH CH CONHCHCONHCH COO3 2 2 2
HOCH C(CH ) COCOO2 3 2
HOCH C(CH ) CH(OH)COO2 3 2
NHCH CH COO2 2HOCH C(CH ) CH(OH)CO3 2)2 3 2
NHCH CH COO2 2OCH C(CH ) CH(OH)CO2 3 2P
CH CH3 2
CH3
CH+
CH(NH )COO3
CH COC(OH)CH CH3 2 3
COO CHCOCOO
CH CH3 2
CH3
NHCHCH SH2
COO NHCH CH CO2 2OCH C(CH ) CH(OH)CO2 3 2P
+ CH SH2
OOCCH(NH )CH CH CONHCHCOO3 2 2
+AdenosylSCH CH CH(NH )COO2 2 3
+SCH CH CH(NH )COO2 2 3
+HO SCH CH(NH )COO3 2 3
+
+S-CH CH(NH )COO2 3
S-CH CH(NH )COO2 3+
HSCH CH(NH )COO2 3
1.1.1.23
H
C
NHNC
HC CH CHCOO2
NHCOCH CH NH2 2 2+
H
C
NHNC
HC CH CH(NH )COO2 3
CHCH CH COO2 2
NHNCH
OC C CH CHCOO
NHNCH
CH
CH COO3
NH
OOC
CH-COOOCNH2 CH2
H NCH CH COO2 2 2
H NCH2 2
CH3CHCOO H NCONHCH CHCOO2 2
CH3
CCO
CH
RPHC N
N
N
HN
C
CCO
CHHCO
N
N
N RP
CCH
N
NH N2
HC
CCO
CH
N
NC
CCH
N
N
RP
H N2
H N2C
O
O
O P~ ~P O
O
O O
P O
O
CH2
N
N
N
N
O
OHOH
NH2
HCCHN
N
N
N O
O
P O O
O
CH2
OH
NH2
HCCH
COOCO
NHOC
NH
CH
H N2 NH2
OOC-CH-CH COO2
HNCO CCH
N
N
C
RPRPH N2
OOCC
CH
NRP
N
CH N2
CH=CHCOO
OH
CH=CHCOO
CH COCOO2
CH2
P
COO
OHO
O
O
COO
O
CH COO2OCH COO2-OOC
OCH2 CH2
OHHC
P
HOCH HCOH
OCCOO
COOHO
OHOHO
NH2COOOCH
NH
CH COCOO2NH
CHO
CO
NH2OH
CH CH(NH )COO2 3
+
N
COO
COO
N+
COO
COORP
O
OH
OH
HO OPPU
COO
O
OH
NHCOCH3
HO
OPPU
COOCH OH2
O
OCCH2 NHAC
HO OPPU
COO
CH OH2
O
OHHO OH
OH
COO
OOCCH N(CH )2 3 2
Dimethylglycine
1.1.99.1
1.2.1.8
COOHO HO
COO
O
HO OH
COO
CH(O ) PPOCH2 COO CH(OH)HOCH2 COOCOO
CH(O )PHOCH2 COO
CH =C(O )2 P COO
CONH2
Ribose -O - P - O - P - O- Adenosine(P)+N
O
O
O
O
+
COO
NRibose - O - P - O - P - O -Adenosine
O
O
O
O
II
IIOH
O
CH CH 2 COO(NH )3+
Epinephrine(Adrenaline)
Norepinephrine(Noradrenaline)
Normetepinephrine(Normetadrenaline)
HOCH CH N(CH )2 2 3 3
+
Adenosyl+
+SCH CH CH(NH )COO2 2 3
CH3
(CH ) N(CH )3 3 2 3CH CH(NH )COO2 3+ +
OH
OOCCH CH CH COSCoA2 2 2
OOCCH CH CH NH2 2 2 2
OOCCH CH CH COCOO2 2 2 OOCCH CH CH CH2 2 2 COOCOO(NH )3
++
HOH
HOH
HHO
HHO
H
Prostaglandin PGE 2
OH
HOCH2 C C CO C COO
OHH
OHH H
-
COO
OR'-CO-OCH
CH O-CO-R2
CH O POCMP2
O
CH CH N(CH )2 2 3+
OHOCH
CH OPO2
CH OH2
O-
CH OPO2
O
CH OCH=CHR2
CH CH N(CH )2 2 3 3+
O-CH OPO2
OR'-CO-OCH
CH O-CO-R23.1.1.32
CH CH N(CH )2 2 3 3+
O-
OHOCH
CH OPO2
CH O-CO-R2
CH CH N(CH )2 2 3 3
+
O-
CH CH(NH )COO2 3+
+C
NHNC
HC
H
CH CH(NH )CHO2 3
HS
11-cis-Retinal
Pi
ADP
NADP+
GDP-Mannuronate
Mannose-6-PDehydro-shikimate
Shikimate
RNA
d-GDP
d-CTP
GUANOSINE-P(GMP)
β-Alanine
P-Ribosyl-AMP P-Ribulosylformimino5-aminoimidazole-carboxamide-R P
Imidazoleacetol-P
HS HSO-
PANTOTHENATE
3-Isopropyl-malate
Succinicsemialdehyde
2.4.1.17
Diacylglycerol
2.3.1.6
1.14.12.1
1.4.4.2
CH CHO3
3.5.4.19
4.2.1.19
3.3.1.1
4.1.1.12
4.1.2.5
COO-2OHCCH
2.1.4.1
2.4.99.7
5.1.3.12
1.1.1.132
4.2.1.46
2.7.1.7
2.4.1.29
2.4.1.21
2.4.1.13
5.4.2.2
2.7.1.6
2.7.7.10
3.1.1.17
4.1.1.34
2.7.1.47
2.7.1.16
ADP
P-Gluconolactone
1.1.1.44
O
OOHHO
OH
CH O2 P
PHENYLALANINETYROSINE
TRYPTOPHAN
INOSINE-P(IMP)
THYMIDINE-P
HISTIDINE
URIDINE-triphosphate
(UTP)
CYSTINE CYSTEINE
ISOLEUCINE
LEUCINE
LYSINE
ORNITHINE
ARGININEHYDROXYPROLINE
PHOSPHATIDYLSERINE Phosphatidyl
inositol
LECITHIN
SPHINGOMYELIN
STEROIDS
Pregnenolone
2.7.6.1
2.4.2.146.3.4.7
5.3.1.9
3.1.3.11
1.2.1.13
2.7.2.3
H
OH OH
C C C
H
CO
OH
C
H
H
OH
POCH2 CH O2 P
5.4.2.1
ADP
4.2.1.11
Endoplasmic Reticulum
1.1.1.100
3.1.1.3
Phosphatidate
2.3.1.39
4.2.1.17
4.2.1.17
4.2.1.55
1.1.1.79
1.2.1.21
1.4.3.8
1.1.1.32
2.7.1.362.7.4.2
4.1.1.33
2.5.1.1
2.5.1.10
2.7.8.5
2.7.8.1
4.1.1.65
2.7.7.14
1.3.99.7
PO
O
CH (CH ) CH=CHCH(OH)CHCH O3 2 12 2
NHAcyl
CH CH N(CH )2 2 3 3+
O-
3.5.1.23
1.14.99.36
2.5.1.32
1.1.1.105
2.4.2.19
1.2.1.32
METHIONINE
4.1.3.27
4.2.1.51
1.14.17.1
1.14.18.1
1.14.13.11
Glycinamide-ribosyl-P
4.2.1.22
3-Oxohexanoyl-CoA
THREONINE
1.5.1.7 - 10
GLUTAMATE
2.3.1.38
ATP
NADH
SUCROSE
ADENOSINE-P(AMP)
TriacylglycerolFAT
RNA
CH3
CH2
OC-COOOH
COO
CH CH 2 COO(NH )3+
GLYCOPROTEINSGANGLIOSIDES
MUCINS
4.1.2.-
2.6.1.-
GLUCOSE
O-Acyl-carnitine
O-Acyl-carnitine
OH
O-
-
R-CO-OCH
R'-CO-OCH
R'-CO-OCH
O HCO-CO-R
CH CH(OH)CH O-P-OCH2 2 2
CH O-CO-R’2
OR'-CO-OCH
O-
CH O-CO-R2
CH O-PO2O-
2.7.8.11
H NCH CH CH CH2 2 2 2 COO(NH )3
+
2.3.1.20 3.1.3.4 2.7.1.107
NH2
+
+H NCNHCH CH CH CH2 2 2 2 COO(NH )3
H
OH HOH
C C C CO
OHH
HOCH2CH OH2
R-CH2COO
CH O-CO-R2
CH O-CO-R"2
R’-CO-OCH
OH
O
CH CHNH2 3+
OR'-CO-OCH
O-
CH O-CO-R2
CH O PO2
COO
OOCCH CH CH2 2 COO(NH )3
+CH CH(OH)CH(NH )COO3 3
O
OHHO
OH
OH
CH OH2CH OH2
NH
CH O-CO-R2
1.3.1.13
4.1.1.28
Malonicsemi-
aldehyde
2.7.2.4
2.1.3.3
3.5.1.26.3.1.2
6.3.
5.5
CarbohydratesBiosynthesisDegradation
Amino AcidsBiosynthesisDegradation
LipidsBiosynthesisDegradation
Purines &Pyrimidines
BiosynthesisDegradation
Biosynthesis DegradationVitamins Co-enzymes & Hormones
Pentose Phosphate Pathway
Photosynthesis Dark Reactions
COMPARTMENTATIONThe "Backbone" of metabolism involves
GLYCOLYSIS in the CYTOPLASM,the TCA CYCLE (mainly) in the Mitochondrial matrix
and ATP FORMATION spanning theMITOCHONDRIAL INNER MEMBRANE
An electron flow (an electric current) generated fromNADH and UQH2 drives the translocation of protons
from the matrix to the intermembrane space.The retrolocation of these protons through the F0 subunits
of ATP synthase to the matrix then supplies the energyneeded to form ATP from ADP and phosphate
Electron Flow Proton Flow
Small Numbers ( eg. 2.4.6.7) refer to the IUBMB EnzymeCommission (EC) Reference Numbers of Enzymes
LEGEND
Human Metabolism is identified as far possible by black arrows
Biosynthesis Degradation
HEMOGLOBIN CHLOROPHYLL
ATP
TR
AN
SL
OC
ATED PROTONS
2.7.1.1
ATP2.7.1.2
3.7.1.2
2.4.1.1
3.2.1.33 3.2.1.23
3.2.1.108
2.7.1.6
2.7.7.12
3.1.3.9
3.2.1.10
2.4.1.25
2.4.2.8
3.5.4.4
1.5.1.3
1.4.4.2
2.6.1.44
4.1.3.4
1.9.3.1
2.5.1.614.1.1.371.3.3.4
1.3.99.3
3.2.1.45
3.2.1.52
3.1.4.12
1.14.16.1
1.14.18.1
1.17.3.2
4.2.1.22
6.4.1.3
1.2.1.24
2.6.1.19
6.3.4.16
2.1.3.3
4.3.2.1
6.3.4.5
5.4.99.2
1.3.99.10
1.2.4.4
1.2.4.4
1.2.4.4
Inborn Errors of Metabolism
AMINO ACID METABOLISM
Fumarylacetoacetase(3.7.1.2)Tyrosinaemia Type I MIM 276700
Phenylalanine hydroxylase(1.14.16.1)Phenylketonuria (PKU) MIM 261600
Tyrosinase(1.14.18.1)Tyrosine Negative AlbinismMIM 203100
Glycine dehydrogenase(1.4.4.2)Non Ketotic Hyperglycinaemia(NKH) MIM 238300
PURINE & PYRIMIDINEMETABOLISM
Xanthine oxidase(1.17.3.2)XanthinuriaMIM 278300
Hypoxanthine guaninephospho-ribosyl transferase(2.4.2.8)HGPRT Deficiency, Primary Gout,Lesch-Nyhan SyndromeMIM 308000
Adenosine deaminase(3.5.4.4)Severe CombinedImmuno-Deficiency (SCID) MIM 102700
FOLIC ACID
Dihydrofolate reductase(1.5.1.3)Megaloblastic Anaemia MIM 126060
ORGANIC ACIDURIAS
Propionyl-CoA carboxylase(6.4.1.3)Propionic Acidaemia MIM 606054
Methylmalonyl-CoA mutase(5.4.99.2)Methylmalonic AcidaemiaMIM 251000
Isovaleryl-CoA dehydrogenase(1.3.99.10)Isovaleric Acidaemia MIM 243500
AMINO ACID METABOLISM
Cystathionine synthetase(4.2.1.22)HomocystinuriaMIM 236200
Branched chain ketoaciddecarboxylase(1.2.4.4)Maple Syrup Urine DiseaseMIM 248600
GABA METABOLISMNEUROTRANSMITTER DEFECTS
Succinic semialdehydedehydrogenase(1.2.1.24)4-Hydroxybutyric AciduriaMIM 271980
4-Aminobutyrateaminotransferase(2.6.1.19)GABA Transferase DeficiencyMIM 137150
UREA CYCLE DEFECTS(HYPERAMMONAEMIA)
Carbamoyl phosphate synthase(6.3.4.16)CPS DeficiencyMIM 237300
Ornithine carbamoyl transferase(2.1.3.3)OCT DeficiencyMIM 311250
Argininosuccinate synthase(6.3.4.5)CitrullinaemiaMIM 215700
Argininosuccinate lyase(4.3.2.1)Argininosuccinate AciduriaMIM 207900
GLYCOGEN STORAGE DISEASES
MUSCLE GLYCOGENOSES
Muscle glycogen phosphorylase (2.4.1.1)
GSD Type V McArdle DiseaseMIM 232600
LIVER GLYCOGENOSES
Amylo-1,6-glucosidase(3.2.1.33)
Glucanotransferase(2.4.1.25)
GSD Type III Cori DiseaseMIM 232400
Glucose-6-phosphatase(3.1.3.9)
GSD Type I Von Gierke DiseaseMIM 232200
MONO- & DI-SACCHARIDES
Sucrase-isomaltase(3.2.1.10)
Sucrose-Isomaltose MalabsorptionMIM 609845
Lactase(3.2.1.23) (3.2.1.108)Lactose Intolerance
MIM 223000
Galactokinase(2.7.1.6)
Galactose Kinase DeficiencyMIM 230200
Galactose-1-phosphateuridyl transferase
(2.7.7.12)Classical Galactosaemia
MIM 606999
PEROXISOMAL DEFECTS
Alanine-glyoxylateaminotransferase
(2.6.1.44)Hyperoxaluria Type 1
MIM 259900
LIPID METABOLISM
Hydroxymethylglutaryl CoA lyase(4.1.3.4)
Ketone Synthesis DefectMIM 246450
Medium chain acylCoA dehydrogenase
(1.3.99.3)MCAD Deficiency
MIM 607008
SPHINGOLIPIDOSES
Acid-β-glucosidase(3.2.1.45)
Gaucher DiseaseMIM 230800
Hexosaminidase A(3.2.1.52)
Tay Sachs DiseaseMIM 272800
Sphingomyelinase(3.1.4.12)
Niemann-Pick DiseaseMIM 257200
RESPIRATORY CHAIN DEFECTS
Cytochrome c oxidase(1.9.3.1)
Complex IVMIM 220110
PORPHYRIAS
Porphobilinogen deaminase(2.5.1.61)
Acute Intermittent PorphyriaMIM 176000
Uroporphyrinogen decarboxylase(4.1.1.37)
Porphyria Cutanea TardaMIM 176100
Protoporphyrinogen oxidase(1.3.3.4)
Variegate PorphyriaMIM 176200
©2006 International Union of Biochemistry and Molecular Biology www.iubmb.org
Designed by Donald Nicholson, The University of Leeds, England – and Sigma-Aldrichin collaboration with Mick Henderson, St.James’ Hospital, Leeds – and the UK MetBioNet Training Group www.metbio.net.
For further information about metabolic disorders visit the OMIM (Online Mendelian Inheritance in Man)web site, hosted by the John Hopkins University – www.ncbi.nlm.gov/entrez/query/fcgi?db=OMIM
GDS
sigma-aldrich.com/pathways
2.3.3.10
