Metabolism
GLYCOGEN STORAGE DISEASES
AMINO ACID METABOLISM
LIVER GLYCOGENOSES
GLYCOPROTEINS
GANGLIOSIDES
MUCINS
O Glycogen synthase 2.4.1.11
2.4.99.7
III Amylo-1,6-glucosidase (debrancher) 3.2.1.33 (Cori)
O
CHOH
CHOH
CH2OH
ACNH
HYALURONIC ACID
COO-
DERMATAN BLOOD GROUP
SUBSTANCES
CHITIN CHONDROITIN
PECTIN
MANNAN
2.4.1.16
CH CH
HO
CHOH
CHOH
CH2OH
ACNH
VI Liver phosphorylase 2.4.1.1 (Hers)
3
CMP-N-Acetyl
COOneuraminate
UDP-N-AcCOOMuramate
2.7.7.43
OH 3.1.3.29
N-Ac-Neuraminate
(Sialate)
MUSCLE GLYCOGENOSES
HO O
5.1.3.6
HO OH
5.1.3.7
CH2OH
O
VII Muscle phosphofructokinase 2.7.1.11
PEP 4.1.3.20
2.7.1.60
ACNH
HO OH
OH
HO OH
5.1.3.14
N-Ac-Mannosamine
COO-
HOCH2
OH
H
OH OH
C
C
C
H
OH H
HO OH
3.1.1.18
OH
H
OH OH
HOCH2
C
C
C
C
H
1.13.99.1
Glucuronate
CO
HOCH2
H
OH
H
OH
C
C
C
H
CO
Gulonolactone
OH
1.1.1.45
Galactokinase 2.7.1.6
HOCH2
C
C
H
OH
HOCH2
UDP-Galactose 4-epimerase 5.1.3.2
OH
C COO-
CO
HOCH2
H
OH
H
OH
C
C
C
H
OH H
HOCH2
HOCH2
Primary sucrase-isomaltase deficiency
Sucrase 3.2.1.48
H
C
C
H
OH OH
HOCH2
HOCH2
HOCH2
C
C
H
OH
CHO
HOCH2
Fructose-1,6-bis-phosphatase deficiency 3.1.3.11
OH
C
C
OH OH
C
C
C
C
OH H
H
C
H
OH
C
H
H
C
C
5.3.1.3
Fructose
CO
C
CH2OH
CO
HOCH2
C
C
H
H
OCH2
P
2.7.1.53
HOCH2
CH2OH
OH
CO
CO
Glucose-6-phosphate dehydrogenase 1.1.1.49
GLUCONEOGENESIS
H
CH2OH
H
OH
OCH2
P
C
C
OH H
C
C
CO
CH2O
CO
C
C
H
H
HOCH2
1.1.1.49
P-Glucono
lactone
Glucose-6-P
CHO
H
C
H
C
H
C
CO CH2OH
2
P
OCH2
H
H
C
C
CO
C
CH2OH
HOCH2
C
OCH2
H
OH
C
C
C
4.1.2.-
CH2OH
OCH2
P
2.7.1.15
H
H
H
C
C
C
CHO
OCH2
P
OH OH OH
P OCH2
2.7.1.19
H
H
C
CO
CH2O
C
C
H
H
H
OH
C
C
C
C
CO
CH2O
CH2
Urea
2.2.1.1
2.7.6.1
HOCH2COCH2O
P
5.3.1
Dihydroxy
acetone-P
4.1.2.13
4.1.2.14
NADP+
P
OCH2
H
C
C
CO
CH2O
HOCH2 C
Ribulose-1:5-di-P
CO2
P
CH2O
Fixation
g-Linolenate
CO.S-ACP
COSCoA
Palmitoleoyl-ACP
Leucotriene B4
O
5.3.99.3
1.14.99.1
Prostaglandin PGE2
CH3(CH2)14CH(OH)CH2COS.CoA
CH3(CH2)14COCH2COS.CoA
Dehydrostearoyl-CoA
OH-Stearoyl-CoA
Oxostearoyl-CoA
Fatty Acyl Synthase - 2.3.1.85 - includes
CH3(CH2)14COSCoA
CH3(CH2)nCH=CHCO.S-ACP
2, 3-Enoyl-ACP
1.3.1.10
Triose phosphate isomerase 5.3.1.1
1.1.1.100
3-OH-Acyl-ACP
4.2.1.61
P
3.5.1.7
2.1.3.2
Formate
3.5.1.8
P
CH3COO-
3, 4-Decenoyl-ACP
2, 3-Decenoyl-ACP
Adenylate kinase 2.7.4.3
CH3(CH2)2CH2CH2CO.S-ACP
Hexanoyl-ACP
CH3(CH2)6CH(OH)CH2COS.ACP
4.2.1.60
3-Hydroxydecanoyl-ACP
CH3(CH2)6CH=CHCOS.ACP
1.3.1.9
4.2.1.60
CH3(CH2)2CH=CHCO.S-ACP
1.3.1.9
3-Oxo-hexanoyl-ACP
1.3.1.9
Butanoyl-ACP
Phosphoglycerate mutase 5.4.2.1
CH3CH=CHCO.S-ACP
6.2.1.3
ACYL-CoA
Pyruvate kinase 2.7.1.40
CH2OH
FATTY ACID
3.1.2.20
CH2OH
HOCH
Acetoacetyl-ACP
CH2O
2.7.1.30
Glycerol
P
CH2O.CO.R
O.CO.R
(CH3)3NCH2CH.CH2COO-
CH2O.CO.R"
3.1.1.3
O-Acyl-carnitine
Triacylglycerol
3.1.1.28
Type I Glycolic aciduria
Alanine: glyoxylate aminotransferase 2.6.1.44
FAT
R.CO.OCH
+
Type II Glycerate dehydrogenase 1.1.1.29
CH2O.CO.R
CH3(CH2)nCH2CH2COSCoA
CH2OH
2.3.1.20
CH3(CH2)nCH=CHCOSCoA
ACYL-CoA
(Mitochondria)
4.1.1.9
CH3CHO
CH3(CH2)2CH2CH2COSCoA
Hexanoyl-CoA
CH3CH2CH2COSCoA
Butanoyl-CoA
Acid lipase 3.1.1.3
CH3(CH2)2CH=CHCOSCoA
1.3.99.3
CH3CH=CHCOSCoA
1.3.99.2
CH3(CH2)2CH(OH)CH2COSCoA
CH3CH(OH)CH2COSCoA
4.2.1.55
Crotonoyl-CoA
3-OH-Butanoyl-CoA
CH3(CH2)3COSCoA
CH3CH2CH=CHCOSCoA
CH3CH2CH(OH)CH2COSCoA
Pentanoyl-CoA
Pentenoyl-CoA
3-OH-pentanoyl-CoA
CH2OR
OH
O
R'OCH
OH
CH2OPO
Long-chain acyl-CoA dehydrogenase 1.3.99.3
4.2.1.52
ROCH
OCH2CH2NH3
Phosphatidyl-glycerol
+
OCH2CH2NH3
P
CH3COCH2NH2
CH2OR
Hydroxymethylglutaryl-CoA synthase 4.1.3.5
CH3COCH2CH2N(CH3)3
Hydroxymethylglutaryl-CoA lyase 4.1.3.4
CH2O P
O-
Choline plasmalogen
Acylsphingosine deacylase (Ceramidase) 3.5.1.23 (Farber)
+
2.3.1.50
Serine
Sphingomyelin phosphodiesterase 3.1.4.12 (Niemann-Pick)
NHAcyl
CH3(CH2)12CH
NH3
CH.CH(OH)CHCH2OH
CH.CH(OH)CHCH2OH
Ceramide
UDP-Sugars
2.4.1.62
Gangliosides
CHCHOHCHCH2O
C 40
Lycopene
hv
NADH dehydrogenase (Ubiquinone) 1.6.5.3
Succinic dehydrogenase (Ubiquinone) 1.3.5.1
1.13.11.21
COO-
Ubiquinol-cytochrome c reductase 1.10.2.2
Opsin
CH3
CH3O
Retinol esters
PORPHYRIAS
5.2.1.3
trans-Retinal
1.2.1.36
11-cis-Retinal
Light
1.1.1.105
CH3
trans-Retinol
(Vitamin A)
HO
CH3
11-cis-Retinol CH2OH
Dark
CH3
Vitamin E
a-Tocopherol
21
12
11
17
24
13
H
HO
CHOLESTEROL
Desmosterol
CH2
Porphyria cutanea tarda (PCT)
Uroporphyrinogen decarboxylase 4.1.1.37
CH
CH3
H
C
N
N
H3C
C
H
N
CH3
H3C
H
C
H2
CH2
-OOC
CH2
H2C
N
H3C
H
C
H2
CH2
H
C
H2
CH2
CH2
CH2
CH2
CH2
CH2
CH2
COO-
COO-
COO-
COO-
COO-
COO-
COO-
COO-
1.3.3.3
Coproporphyrinogen
Uroporphyrinogen
4.1.1.37
III
III
COO-
P
CH2
CHO
CH.COO-
-OOC
CH2
H2C
CH2
H2C
4.3.1.8
4.2.1.75
Red Cytochrome-c
ADP+Pi
4.2.1.24
6.3.4.5
2.1.3.3
P
+
1/2
O2
NHCOCH3
OHCCH2CH2CHCOO-
2.7.2.8
N-Acetylglutamate 1.2.1.38
Ox
ATP
1.9.3.1
H2N
Porphobilinogen
Erythropoietic Protoporphyria
Ferrochelatase 4.99.1.1
NHCOCH3
1.14.13.39
NH
HN
N(CH3)CH2
Creatinine
H 2O
O
CO
C
-O
H2NCH2CH2CH2CHCOO-
2.6.1.11
P
NO
P-creatine
+
+
ARGININE
+
2.7.3.2
3.9.1.1
4.3.2.1
H2NCNHCH2CH2CH2CH(N H3)COO+
NH2
NH2
H2NCN(CH3)CH2COO-
Creatine
2.1.1.2
H2NCNHCH2COO-
+
CH2(N H3)COO-
Glycocyamine
Guanidoacetate
2.1.4.1
GABA transaminase deficiency
4-Aminobutyrate aminotransferase 2.6.1.19
+
+
4-Hydroxyglutamate
Hyperprolinaemia I
Proline dehydrogenase 1.5.99.8
Spermine
2.5.1.22
1.2.1.26
Hyperprolinaemia II
Pyrroline-5-carboxylate reductase 1.5.1.2
OHCCH2CH2COCOO3.5.4.22
2 Oxoglutarate-4semialdehyde
OHCCOO-
4-Hydroxy2-oxoglutarate
UREA CYCLE
Glyoxylate
-OOCCH(OH)CH COCOO2
2.6.1.23
Aminobutyrate deaminase 4.1.1.15
H2N(CH2)3NH(CH2)4NH(CH2)3NH2
C.COO-
4-Hydroxypyrroline2-carboxylate
-OOCCH(OH)CH CH(N H )COO2
3
Carbohydrates
NH2
3.5.3.1
+
(CH3)3N (CH2)3CH2CH(N H3)COO-
CH3COCOO4.1.3.16
Pyruvate
Citrullinaemia
Argininosuccinate synthase 6.3.4.5
Legend
3.5.1.16
N-Acetylornithine
+
NH2
NHCN(CH3)CH2COO-
O3.5.2.10
4-Hydroxybutyrate dehydrogenase 1.1.1.61
CH2
CH2
CH.COO-
UREA
NHCOCH3
N-Acetylglutamic
semialdehyde
Acetyllysine
1.4.3.2
2.6.1.65
N
3-Hydroxypyrroline- 1.5.1.12
5-carboxylate
Argininosuccinate
3.5.3.6
ORNITHINE
Spermidine
HOCH
CH2
CH
N
H2NCH2CH2CH2CH(N H3)COO2.6.1.13
Putrescine 2.5.1.16
N
CITRULLINE
+
H2N(CH2)4NH(CH2)3NH2
HYDROXYPROLINE
HOCH
H2NCONH2
ADP+Pi
Cytochrome
oxidase
N
H
PROLINE
1.5.1.2
H2NCNHCH2CH2CH2CH(N H3)COO-
H2N
+
CH3CONH(CH2)4CH(N H3)COO-
N6-Trimethyl- 1.14.11.8 N6-Trimethyllysine
4.1.2.3-OH-lysine
4.1.1.50
S-Adenosylmethylthiopropylamine
Decarboxylated S-adenosylmethionine
N
H
1.14.11.2
Carbamyl-P
Glutamic semialdehyde
2-Oxo-6-acetyl
aminohexanoate
CH.COO-
H2C
CH.COO-
+
CH2
2.3.1.32
CH3CONH(CH2)4COCOO-
CH2
HOCH
HOOCCHCH2COO-
OOCCH2CH2CH(N H3)COO-
Glutamyl-P1.2.1.41
1.10.2.2
2H+
4-Aminobutyraldehyde
NH
1.5.99.8
3.5.1.20
+
Red
-OOCCH CH CH.COO2
2
-OOCCH CH COCH NH +
2
2
2
3
5-Aminolevulinate
COO-
CH2
Protoporphyrinogen
IX
ATP
CH3
CH2
1.3.3.4
4.99.1.1
Ubiquinone
H2N.CO.O
Hyperlysinaemia
Saccharopine dehydrogenase (NADP, lysine-forming) 1.5.1.8
Saccharopine dehydrogenase (NADP, glutamate-forming) 1.5.1.10
1.5.1.10
1.5.1.9
(CH3)3N(CH2)3CH(OH)CH(N H3)COO-
H2NCH2CH2CH2CH2NH2
CH2
H2NCONHCH2CH2CH2CH(N H3)COO-
2.7.2.11
1.3.5.1
Ox
Pyrolline-5carboxylate
4.1.1.17
6.3.4.16
NH4
1.6.5.3
+
CH2
CH.COO-
5.4.3.8
N
CH2
ATP
+
1.18.6.1
1.19.6.1
2.3.1.35
CH2(NH3 )COO-
CH2
CH2
CH2
Ox
2.3.1.37
CH2
HEME
ADP+Pi
Glycine
N
H
H
H2C
-OOC
Red
7
6
N2
COO-
CH2
N
CH2
5
Squalene
C 30
CH2
CH2
N
6.3.5.5
+
+
+
4-Trimethylaminobutyraldehyde
1.2.1.19 H2NCH2CH2CH2(CHO)
4-Aminobutyrate
Glutamine
Saccharopinuria
Saccharopine dehydrogenase 1.5.1.9
LYSINE
Saccharopine
2-Oxo-6-aminohexanoate
(CH3)3N (CH2)3CHO
1.2.1.47
+
1.6.6.4
Enz- Fe-S
CH2
H2C
N
CH3
CH2
H2
C
N
H
CH2
H
COO-
CH2
N
H
H2C
N
CH3
CH3
H2
C
N
H
CH2
H
5.4.99.7
1.14.99.7
COO
COO-
H3C
N
H
H2C
N
CH2
Lanosterol
-
CH2
CH2
Variegate Porphyria (VP)
5-Aminolevulinate synthase 2.3.1.37
Protoporphyrinogen oxidase 1.3.3.4
COO-
CH2
CH
N
H
CH
CH3
H2
C
H
Zymosterol
CH2
H3C
N
Fe
HC
CH
CH2
CH
HO
H
CH2
H3C
Hereditary coproporphyria (HCP)
Coproporphyrinogen oxidase 1.3.3.3
H
HO
1.14.11.1
+
CH
ATP CO2
1.6.5.3
1.6.99.2
15
14
8
4
H
25 27
16
9
1
3
+
(CH3)3N (CH2)3COO-
-OOCCH CH CH N H
2
2
2
3
1.7.7.1
NO2
Glutaric aciduria (Type I)
Glutaryl-CoA dehydrogenase 1.3.99.7
CHCH2CH2COOH2NCH2CH2CH2CH2CH(N H3)COO+
1.5.1.7
CH2CH2CH2CH2CH(N H3)COO1.5.1.8
H2NCH2CH2CH2CH2COCOO-
4-Trimethylaminobutyrate
1.5.1.2
23
2-Aminoadipate
1.2.1.31
semialdehyde
Piperideine-2carboxylate
2.6.1.19
3.5.1.2
6.3.1.2
1.4.1.2
COO-
N
1.5.1.1
1.5.1.21
-OOCCH CH CH OH
2
2
2
4.1.1.15
H2NCO.CH2CH2CH(N H3)COO-
1.6.6.1-3
COO-
Pipecolate
+
OHCCH2CH2CH2CH(N H3)COO-
4-Hydroxybutyrate
+
-
26
2
HO
1.7.99.4
Red
22
20
Acute intermittent porphyria (AIP)
Porphobilinogen deaminase 4.3.1.8
Congenital erythropoietic porphyria
Uroporphyrinogen III synthase 4.2.1.75
Enz- FMN
Ox
2.5.1.21
O
Oxopantoate
Glutaryl-CoA
1.1.1.61
1.5.1.12
NO3
N
H
1.5.99.3
-OOCCH CH CH COSCoA
2
2
2
CH2
1.6.5.3
1.6.99.2
Farnesyl-PP
C 15
Succinic
semialdehyde
2-Aminoadipate
1.1.1.155
+
-OOCCH CH CHO
2
2
1.4.1.13
Hyperpipecolic acidaemia
Pipecolate dehydrogenase 1.5.99.3
CH3
1.1.1.169
HOCH2 — C — CO — COO-
NH
+
-OOCCH CH CH CH(N H )COO2
2
2
3
2-Oxoadipate
Carnitine
-
Isovaleryl-CoA
CH3
4.1.2.12
NHCOCH2CH2COONHCOCH2CH2COO+
N H3
-OOCCH.CH CH CH CHCOO2
2
2
-OOCCHCH CH CH CHCOO+
2
2
2
N H3
+
2.6.1.17
N H3
CH.COO-
-OOCC
HCHO
-OOCCOCH CH CH CH.COO2
2
2
CH2
N
(CH3)3N CH2CH(OH)CH2COO-
GLUTAMATE
ELECTRON
TRANSFER
SYSTEM
2.5.1.10
Vitamin K
1.2.1.16
-OOCCH CH CH(N H )COO2
2
3
CH2OH
O
1.3.99.7
+
NADH
OPP
Phytol
C 20
1.1.1.105
5.2.1.7
CHCH2OPP
Geranyl-PP
C 10
CH2
CHO O
CH2OH
2.3.1.76
3.1.1.21
Porphobilinogen synthase deficiency 4.2.1.24
CHCH2CH2C
H2
C
H2C
CH.COO-
COO-
N
4.1.1.71
Pantoate
(CH3)2CHCH2COSCoA
1.3.99.10
Aminoadipic aciduria
2-Aminoadipate aminotransferase 2.6.1.39
3.5.1.22
6.3.2.1
HOCH2C (CH3)2CH(OH)COO-
2.6.1.6
CH2
-OOHCCH CH CH COCOO2
2
2
2.6.1.39
Homoisocitrate
2.6.1. -
CH3
Geranyl-geranyl-PP
C 20
n
Coenzyme Q
Ubiquinone
O
CHO
Cytochrome c oxidase 1.9.3.1
CH3C
CH2OPP
O
LEUCINE
Oxoleucine
N
CH(OH)COO-
R.CO.COO-
2-OXO ACID
PANTOTHENATE
ß-Alanine
COO-
Piperideine-6carboxylate
TRANSAMINATION
2.7.1.33
+
CH.COO4.2.1.36
Homocitrate
Asparagine
+
R.CH(N H3)COO-
H
C
4-Hydroxybutyric acidaemia
Succinic semialdehyde dehydrogenase 1.2.1.16
4-P-Pantothenate
HOCH2C (CH3)2CH(OH)CONHCH2CH2COO-
CH2CH2COO-
CH2COO-
OCH2C (CH3)2CH(OH)CONHCH2CH2COO-
P
Isobutyryl-CoA
(CH3)2CHCH2COCOO- (CH3)2CHCH2CH(N H3)COO-
3-Methylcrotonyl-CoA
6.3.2.5
Aspartyl
2, 3-Dihydro- 1.3.1.26 Piperideine-2, 6N-Succinyl-2N-Succinyl-2,6Diamino4.2.1.52
semialdehyde
dipicolinate
dicarboxylate amino-6-oxopimelate diaminopimelate 3.5.1.18 pimelate
HO–CCOO4.1.3.21
2-AMINO ACID
NAD+
CH3
CH3CHCO.SCoA
1.3.99.3
Isovaleric acidaemia
Isovaleryl-CoA dehydrogenase 1.3.99.10
4-P-Pantothenylcysteine
1.2.1.25
Cysteine
CH3C = CHCOSCoA
-OOCC
+
CH2CH2COO-
5.1.99.1
OCH2C(CH3)2CH(OH)CONHCH2CH2CONHCHCH2SH
CH3
OHCCH2CH(N H3)COO1.2.1.11
P
VALINE
CH2 = CCOSCoA
3-Methylcrotonylglycinuria
Methylcrotonyl-CoA carboxylase 6.4.1.4
4.1.1.36 COO-
+
MethylacrylylCoA
3-Isopropyl1.1.1.85
malate
HC
H2NCOCH2CH(N H3)COO-
2-OXOGLUTARATE
2.5.1.1
CH3
2.5.1.32
Rhodopsin
CH3O
Retinoate
CHCH2OPP
COOH
(CH3)2CHCHCH(OH)COO-
Hypervalinaemia
Valine aminotransferase 2.6.1.32
2
OCH2C(CH3)2CH(OH)CONHCH2CH2CONHCH2CH2SH
1.2.1.25
COOH
2
4-P-Pantetheine
CHCH(N H3)COO-
CH3
(CH3)2CHC(OH)CH2COO-
2
2.7.7.3
P
2.6.1.32
6.4.1.4
Aspartyl-P
2.7.2.4
6.3.5.4
5.4.99.2
1.2.4.2
Isopentenyl-PP
C 5
2.5.1.29
-OOCCH CH(N H )COO2
3
-OOCCH CH COSCoA
2
2
-OOCCOCH CH COO2
2
CH3
2.6.1.32 CH3
4.2.1.17
2
2 Methylbutyryl
CoA
CH3
3-Methylglutaconyl-CoA
OOCCH2CH(N H3)COO-
3 2
Dephospho-Coenzyme A
1.3.99.3
2-Oxoisovalerate
4.2.1.9
3-Hydroxyisobutyryl
CoA
4.2.1.33
2
CH3CH2CHCOSCoA
CHCOCOO-
CH3
+
P
ASPARTATE
SUCCINYL-CoA
1.1.1.41
CH2
Dimethylallyl-PP
C 5
Lumirhodopsin
ß-CAROTENE
C 40
Metarhodopsin
GDP+Pi
CO2
OH
O- O-
CH3
HOCH2CHCOSCoA
2-Isopropylmalate
[ O.P.P.-Adenosine
CH C(CH ) CH(OH)CONHCH CH CONHCH CH SH
+
CH3
Tiglyl-CoA
-OOCCH C = CHCOSCoA
2
6.2.1.4
3.1.2.3
CH3C.CH2CH2OPP
CH3C
Phytoene
C 40
O
4.1.1.33
Galactose
CH3
RESPIRATORY CHAIN
GTP
2-3-dihydroxy
isovalerate
CH3
2.7.1.24
CH(N H3)COO-
CH3
Maple Syrup Urine
Brached -chain oxo-acid dehydrogenase 1.2.1.25
Coenzyme A
1.1.1.3
CH3CH2
CH3
3.1.2.4
CH3
CHCOCOO-
CH3CH = CCOSCoA
C(OH)CH(OH)COO-
HOCH2CHCOO-
6.4.1.3
+
Cerebroside
3.2.1.46
2.4.1.47
3.2.1.45
4.1.3.1
ISOCITRATE
CH3C(OH)CH2CH2OP.O.P
NHCOR
CH3(CH2)12CH
O
DiphosphoMevalonate
Psychosine
Acyl-CoA
NHCOR
CH3(CH2)12CH
CH2COO-
2-Acetolactate
CH2C(CH3)2CH(OH)CONHCH2CH2CONHCH2CH2SH
-OOC.CH.COSCoA
+
SUCCINATE
CH2COO-
CHOLINE
CHCH(OH)CHCH2O Galactose
CH3(CH2)12CH
4-Sphingenin
2.4.1.23
Acyl-CoA 3.5.1.23UDP-Galactose
+
CHCH(OH)CHCH2OPOCH2CH2N(CH3)3
3.1.4.12
O2.7.8.3
HOCH2CH2N(CH3)3
NH3
+
CH3(CH2)12CH
SPHINGOMYELIN
Glucosyl-ceramidase 3.2.1.45 (Gaucher)
2.7.1.32
+
3.1.4.4
Sphinganin
1.1.1.102
Choline-P
-OOC.CH CH COO2
2
CHCOO-
2.7.1.36
2.7.4.2
+
P OCH CH N(CH )
2
2
3 3
2.7.7.15
3.1.4.3
NH3
CH3(CH2)14CH(OH)CHCH2OH
Dehydrosphinganin
+
+
CPP OCH2CH2N(CH3)3
CDP-choline
2.7.8.2
+
1.3.1.35
NH3
CH3(CH2)14COCHCH2OH
Galactosyl ceramidase 3.2.1.46 (Krabbe)
OCH2CH2N(CH3)3
LECITHIN
3
Methylmalonyl-CoA
D L (B12)
CHOHCOO-
Mevalonate
+
+
CH2OPOCH2CH2N(CH3)3
O-
SPHINGOLIPIDOSES
O
R'OCH
O
ROCH
4.3.1.1
1.3.5.1
CH3C(OH)CH2CH2OH
2.3.1.6
+
Bile Acids
Homoserine
[ O.P.P.-Adenosine-3´- P
2-Oxo-3-methyl
ISOLEUCINE
2.6.1.42
valerate
4.2.1.17
Methylmalonic acidaemia
Methylmalonyl-CoA decarboxylase 4.1.1.41
Methylmalonyl-CoA mutase 5.4.99.2
Methylmalonyl-CoA epimerase 5.1.99.1
CH2SH
Glutathione
HOCH2CH2CH(N H3)COO2.3.1.46
O-Phosphohomoserine
CH3CH2
CH3
CH3
+
OCH2CH2CH(N H3)COO-
4.2.99.2
CH3CHOHCHCOSCoA
Erythrocyte glutathione synthase deficiency
Glutathione synthase 6.3.2.3
Hydroxyisobutyryl-CoA hydrolase 3.1.2.4
+
S-Adenosyl
methionine
(SAM)
2.1.1.10
6.3.2.3
+
CH3
+
SCH2CH2CH(N H3)COO-
-OOCCH(N H )CH CH CONHCHCONHCH COO3
2
2
2
CH2SH
g-Glutamylcysteine
P
1.2.1.25
2.5.1.6
Adenosyl
CH3
2.1.1.20
Propionic acidaemia
Propionyl-CoA carboxylase 6.4.1.3
Glycine
CH3CHOHCH(N H3)COO-
THREONINE
C(OH)CH(OH)COO-
2.1.1.14
-OOCCH(N H )CH CH CONHCHCOO3
2
2
Taurine
CH3CH2
CH3COC(OH)CH3
CH3
CH2COO-
3.1.4.2
Glycerophosphocholine
2.1.3.1
4.1.1.41
5.1.99.1
4.2.1.3
1.1.99.1
+
CH2OPOCH2CH2N(CH3)3
O3.1.1.5
1.8.1.3
CH3
Propanoyl-CoA
1.4.3.1
-OOCCH=CHCOO-
FUMARATE
Acetylcholine
+
Lysolecithin
CH2OR
4.2.1.2
Glyoxylate
4.2.1.18
Histidinal
METHIONINE
+
6.3
.2.2
HO3SCH2CH2NH2
2-Aceto-22:3-diHydroxyhydroxy-3 4.2.1.9
butyrate 1.1.1.86 methylvalerate
CH3CH2COSCoA
Hypermethioninaemia
Methionine adenosyltransferase 2.5.1.6
CH2CH(N H3)CHO
NH
+
S-Adenosyl
homocysteine
Glutamate
CH3
3-Hydroxyisobutyrate
4.1.3.2
HOOCCHO
CITRATE
1.1.1.32
+
O
HOCH
CH2OPOCH2CH2N(CH3)3
O-
2.1.1.17
2.1.1.71
CHR
Ethanolamine
CH2OH
O
HOCH
CH2OCH
Mevaldate
Cysteate
1.1.1.103
Aminoacetone
1.1.1.86
MALATE
CH2COO-
+
C
CH3SCH2CH2CH(N H3)COO2.1.1.13
3.3.1.1
+
-OOCCH(OH)CH COO2
C(OH)COO-
Glutamylcysteine synthase deficiency
Glutamate-cysteine ligase 6.3.2.2
C
H
1.1.1.23
+
CH2CH2CH(N H3)COO-
1.1.1.37
4.1.3.7
CH2COO-
CH2COO-
2.7.7.14
2.7.8.1
4.1.3.8
CH3C(OH)CH2CHO
1.4.3.8
+
HOCH2CH2NH3
2.7.1.82
Ethanolamine-P
CDP ethanolamine
Phosphatidyl
ethanolamine
CEPHALIN
Glycol
aldehyde
CH2OPOCH2CHOHCH2OH
O-
+
CPP
O
1.1.1.34
HOCH2CHO
Histidinol
1.1.1.23
N
SCH2CH2CH(N H3)COO-
HOOCCH2CH2CO.O
1.1
.1.3
1
3.1.3.15
CH2CH(NH2)CH2OH
NH
+
+
2-Methyl-32-Methyl
1.1.1.35
Hydroxyacetoacetyl-CoA
butyryl-CoA
CH
COO-
-OOCCOCH COO2
Histidinol-P
C
HC
Homocysteine
HO3SCH2CH(N H3)COO-
Hypotaurine
COO-
OXALOACETATE
Homocysteinaemia
Cystathionine synthase 4.2.1.22
5-Methyltetrahydrofolate-homocysteine
methyltransferase 2.1.1.13
P
CH2CH(N H3)COO-
HSCH2CH2CH(N H3)COO-
4.4.1.8
HO2SCH2CH2NH2
4.1.1.32
4.1.3.4
CH2CH(NH2)CH2O
P
C
H
N
4.2.99.9
OHCCHCOO-
ß-OH-ß-Methylglutaryl-CoA
1.2.1.21
Oxalate
4.2.1.22
+
SCH2CH2CH(N H3)COO-
Cystathionine
4.1.1.29
Oxobutyrate
C
NH
N
HISTIDINE
4.3.1.3
Adenosyl
Methylmalonyl
semialdehyde
1.4.1.10
2.6.1.4
2.6.1.44
CH3C(OH)CH2COSCoA
HOCH2COO-
Glycolate
HOOC.COOH
CH2OR'
O
1.2.3.5
4.2.1.49
Imidazole acetol-P
+
C
C
H
CH2COCH2O
NH
C
H
HISTAMINE
N
C
2.6.1.9
HC
HC
HC
Histidinaemia
Histidine ammonia-lyase 4.3.1.3
4.2.1.19
CH2CH2NH2
NH
N
HN
C
H
NH
Urocanate
Cystathioninuria
Cystathionine lyase 4.4.1.1
CH
C
OH OH
4.1.1.22
CH
C
+
4.4.1.1
+
CH3
-OOCCHO
CH2COO-
C
H
C
C
H
CHCOO-
RPPP
Imidazole glycerol-P
HC
C
H
H
RP
N
C
CH
HO2SCH2CH(N H3)COO-
CH3CH2COCOO-
OCH2
N
HC
AMINO ACIDS
CH
OC
CTP
N
N
Imidazole
acetaldehyde
Imidazole
acetate
CH2CH(N H3)COO-
CH3COC(OH)CH2CH3
6.4.1.1
Glyoxylate
N
CYSTEINE
4.1
.3.1
8
2.3.1.16
4.1.3.5
NH
CH
Imidazolone
3.5.2.7
propionate
+
1.2.1.18
ACETYL-CoA
2.3.1.9
1.1.3.15
1.1.1.79
CDP-diacyl
glycerol
Inositol
Cardiolipin
+
Acetyl-CoA C-acyltransferase 2.3.1.16
N
C
HC
P
CH
NH
Hydantoin-5propionate
CH
CONH2 N
C
NH
CH2
C
H
C
O
OC
CO
N
N
6.3.4.2
UTP
C — CH2CHO
HC
N
CHCH2CH2COO-
3.6.1.15
2.7.4.6
Glutamate formiminotransferase 2.1.2.5
NH2
C
CH
CH
CH
P-Ribulosylformimino
5-Aminoimidazolecarboxamide-RP
N
+
4.1.3.18
3-Oxopentanoyl-CoA
2.7.8.11
CH2OPOCH2CHOHCH2OPOCH2
OO-
CH2OP OCH2CH2NH3
O-
3-Hydroxyacyl-CoA dehydrogenase 1.1.1.35
NH
HSCH2CH(N H3)COO-
CH3COCHO
CH3CH2COCH2COSCoA
1.1.1.35
CH2OPPC
HCOR
O
ROCH
4.1.1.65
O
R'OCH
2.7.8.8
R'OH2C
CH2OR'
CH2OR
Short-chain acyl-CoA dehydrogenase 1.3.99.2
Serine
PHOSPHATIDYL
SERINE
OH
Medium-chain acyl-CoA dehydrogenase 1.3.99.3
R'OCH
OHO OH
Phosphatidyl
inositol
CHCH2CH2COO-
HN
Formimino
glutamate
1.6.4.1
Methylglyoxal
1.2.1.23
Acetoacetyl-CoA
2.7.7.41 2.7.8.5
3.1.3.27
CH2OR
COO-
O
ROCH
+
CH2OPOCH2CHNH3
O-
OC
CH3COCHCOSCoA
CH2OR
Pancreatic lipase 3.1.1.3
CHCH2CH2COO-
H
C
5.3.1.16
NH
C
H
Imidazolone
acetate 1.14.13.5
CH
4.2.99.8
Odd C Fatty acids
Triacylglycerol lipase 3.1.1.3
N
H
OH OH
C — CH2COO-
HC
O
C
RPPP
2.7.4.6
UDP
P OCH2C
RPP
Dihydrofolate reductase 1.5.1.3
CDP
N
2.7.4.4
N
N
5,10-Methylenetetrahydrofolate reductase 1.7.99.5
d-CDP
1.17.4.1
HN
CH
C
HC
2.7.4.14
d-CMP
2.4.2.1
OC
RP
FOLIC ACID
2.7.4
.6
CH
N
P-Ribosylformimino
5-Aminoimidazolecarboxamide-RP
NH
NH
HN
2.1.2.5
NH2
OC
NH C
3.5.4.12
CH
Uridine-P
(UMP)
Orotic aciduria
Orotate phosphoribosyltransferase 2.4.2.10
Orotidine-5´-phosphate decarboxylase 4.1.1.23
RP
GUANOSINE-P
(GMP)
d-CTP
Cytosine
Succinylhomoserine
Malonic
semialdehyde
LACTATE
CH3COSCoA
2.3.1.16
CH3COCH2COSCoA
1.1.1.157
C
4.1.1.12
CH3CH(OH)COO-
1.1.1.39
CH3(CH2)2COCH2COSCoA
3-Oxohexanoyl-CoA
H
C
OHCCH2COO-
4.1.1.12
1.1.1.27
ATP
GTP
2.3.1.38
3-Oxoacyl-CoA
1.1.1.35
3-OH-Hexanoyl-CoA
H
C
NH
CHCH2CH2COO-
3-Sulphinyl
pyruvate
+
CH3CH(N H3)COO-
ALANINE
2.3.1.16
4.2.1.17
2, 3-Hexenoyl-CoA
H
C
Carbamoyl glutamate
HS-
OC
4.1.1.23
N
DP
CH
N
H
CHCH2COOC
H
O
C
NH
6.3.4.1
6.3.5.2
CH
C
2.7.4.4
N
H
3.5.4.1
C
N
CH
OC
H
Thymine-uraciluria
Dihydropyrimidine dehydrogenase 1.3.1.2
RP
N
C
CH
d-UMP
2.1.1.45
2
C
N
N
XANTHOSINE-P
(XMP)
O
CH
OC
2.7.7.6
N
Uracil
2.4.2.9
OH OH
O
OC
2.7.7.6
DP
CH
N
RP
O
C
CH
C
N
H
H2NC
GDP
2.7.4.6
HN
C.CH3
CH
OC
1.3.1.2
CH
OC
C
O
-OOC
C
HN
d-GDP
GTP
3-Aminoisobutyric aciduria
3-Aminoisobutyrate aminotransferase 2.6.1.22
N 1.1.1.205
OC
HN
THYMIDINE-PNH
O
C
HN
N
N
HO2SCH2COCOO-
2.6.1.2
d-GTP
CH
N
2.4.2.4
Orotidine-P
OCH2
HN
C.COO-
N
4.1.2.5
Acetaldehyde
LIPID METABOLISM
Acylcarnitine hydrolase 3.1.1.28
O
C
HN
O
C
OC
Cysteine sulphinate
CH3CO.S-ACP
CH3(CH2)nCOCH2COSCoA
RNA
2.7.4.9
Dihydrouracil
P
NH
Cysteamine
1.4.1.1
1.2.4.1
2.3.1.12
1.8.1.4
RP
2.4.2.15, 3.5.4.3
1.13.11.20
Acetyl-ACP
3.1.2.11
2.8.3.5
3-OH-Acyl-CoA
4.2.1.17
Malonyl-Co-A
Acetoacetate
Phosphatidate
3.1.3.4
2.7.1.107
PYRUVATE
CH3COCH2COO-
CH3(CH2)nCH(OH)CH2COSCoA
1.1.1.35
2, 3-Enoyl-CoA
1.3.99.3
4.1.1.4
CH2O P
Diacyl
glycerol
1.1.1.30
NH
N
2.7.7.6
Adenylosuccinate lyase 4.3.2.2
RP
INOSINE-P
(IMP)
.4.4
O
C
Adenylosuccinate
2.7.7.7
RP
CH 3.5.4.10
N
CH
C
1.17.4.1
CH2
3.5.4.19
H2N
C
3.5.4.6
4.1.1.29
HOOCCH2CO.SCoA
C
HC
4.3.2.2
Primary Gout
I AMP deaminase 3.5.4.6
II Hypoxanthine phosphoribosyltransferase 2.4.2.8
III Ribosephosphate pyrophosphokinase 2.7.6.1
CH
N
N
C
N
RP
HSCH2CH2NH2
4.1.1.1
6.3
NH
N
Formylamidoimidazolecarboxamide-RP
N
HC
2.7.7.7
CH2
RPPP
-OOC
2.7.7.4
2.7.1.25
+
4.4.1.1
3.7.1.2
HN
-OOC.CH.CH COO2
CH
N
DNA
2.7.7.6
C.CH3
2.4.2.10
Formimino
aspartate
S — CH2CH(N H3)COO-
3
CH3COCOO-
ACETONE 3-OH-Butyrate
CH2O.CO.R
2.3.1.15
2.3.1.51
R.CO.OCH
R.CO.OCH
2.3.1.39
CH3CH(OH)CH2COO-
CH3COCH3
O
C
Orotate
C
H
1.1.1.1
KETONE BODIES
3-P-Glycerol
C
.1.1
ATP
Malonyl-ACP
2.7.7.7
Thymine
C.COO-
CHCH2COO-
HN
O
C
N
Fumarate
(AMP)
CH
N
H
CH
C
3.6.1.31
CYSTINE
2.3.1.41
2.3.1.7
Carnitine
HOOCCH2CO.S-ACP
O
C
P-Ribosyl-AMP
-OOC
+
4.2
1.1.1.8
HOCH
CH2OH
(Cytosol)
Hyperoxaluria
ETHANOL
2.7.1.40
Hypoxanthine
ADENOSINE-P
N
H
N
N
S — CH2CH(N H3)COO-
CH3COCH2CO.S-ACP
1.1.1.100
3-OH-Butanoyl-ACP
R.CH2COO-
CH3CH2OH
ADP
CH3CH(OH)CH2COS-ACP
4.2.1.58
Crotonoyl-ACP
CH3(CH2)n+2CO.S-CoA
)COO-
2.3.1.41
Phosphoglycerate kinase 2.7.2.3
CH3CH2CH2CO.S-ACP
2.3.1.30
P-enolpyruvate
CH3(CH2)2COCH2COS.ACP
1.1.1.100
P
1.3.1.2
N
H
C
HC
1.8.99.1
3-Oxo-decanoyl-ACP
CH2 C(O
3-Hydroxyhexanoyl-ACP
2
C
N
HSO3-
2.3.1.41
CH3(CH2)2CH(OH)CH2CO.S-ACP
4.2.1.59
2, 3-Hexenoyl-ACP
C
RP
Asparate
2.4.2.1
2.4.2.8
NH
2.7.7.7
1.8.99.4
CH3(CH2)6COCH2COS.ACP
1.1.1.100
C
P-Adenyl-SO4
ACETATE
2.3.1.41
CH3(CH2)5CH=CHCH2COS.ACP
CH3(CH2)6CH2CH2COS.ACP
Decanoyl-ACP
H
N-Formyl
aspartate
1.8.2.1
1.8.3.1
4.2.1.11
Glyceraldehyde-3-phosphate dehydrogenase 1.2.1.12
H
SO42-
2.7.2.1
3-Oxoacyl-ACP
OC
CH
OC
C
3.5.3.5
COO-
O
C
1.3.1.14
Dihydro
orotate NH
H
NHCHO
CH3(CH2)nCOCH2CO.S-ACP
HN
3.5.2.2
HN
N
H
-OOCCHCH COO2
ACETYL-P
P
CH.CH3
CH2
Dihydro
thymine
C.COO-
P-Ribosyl-ATP
5.4.2.1
2-P-Glycerate
3.5.2.2
N
H
Carbamoyl
ß-alanine
OH OH
O
2.4.2.17
HCOO-
CH3COO
OC
H2NCONHCH2CH2COO-
+
C
OCH2
P
P-hydroxypyruvate
O
C
HN
CH2
OC
3.5.2.3
Carbamyl
aspartateH
Glycerate
)COO-
HOCH2CHO
CH3(CH2)nCH(OH)CH2CO.S-ACP
4.2.1.60
OCH2CH(O
Mitochondrial
Chain elongation
Palmitoyl-CoA
P
2, 3-Diphosphoglycerate
Microsomal
EC 2.3.1.38, 2.3.1.39, 2.3.1.41, 1.1.1.100, 4.2.1.61, 1.3.1.10, and 3.1.2.14
CH3(CH2)14COS-ACP
Palmitoyl-ACP
1.3.1.9
OH
Thromboxane B2
CH3(CH2)14CH=CHCOSCoA
1.1.1.95
2.7.1.31
HOCH2CH(OH)COO-
O
HO
5.3.99.5
OH
HO
COO-
O
C
HN
CH.COO-
OC
N
2.6.1.22
H
P OCH2COCOO-
3-P-Glycerate
O
COO-
ß-Ureido
isobutyrate
3.5.1.6
-OCO
CH2
H2N
2.6.1.52
OCH2CHOHCOO-
CH
C
N
d-ATP
2.7.4.6
HN
H2NCH2CH2COO-
Phosphoserine
ATP
OH OH
COO-
1.13.11.34
Arachidonate
.6
3.5.1
ß-Alanine
P OCH2CH(NH2)COO-
1.1.1.29
1.1.1.81
N
2.4.2.1
OC
4.1.1.11
3.1.3.3
2.6.1.18
COO-
COSCoA
HOCH2COCOO-
2.7.2.3
OPP
HO
CH3
ß-NH2-Isobutyrate
O
NH2 C C
HCO
N
2.1.2.3
H
CH
4.3.2.2 NH2
1.17.4.1
CH3
1.4.1.7
Hydroxypyruvate
ADP
P
O
P-Ribosyl-PP
4.1.1.39
OCH2CHOHCOO P
1:3-bis-P-Glycerate
C
N
HC
2.7.4.3
Xanthinuria
Xanthine oxidase 1.1.3.22
Hippurate
N
C
C
OH
H2NCONHCH2CHCOO-
CO.NHCH2COO-
1.14.13.12
N
HC
NH 1.1.3.22
ADP
OH
O
C
HN
CH
C
TDP
H2NCH2CHCOO-
CHO
OH
Glyceraldehyde
C
N
H
2.7.4.6
SERINE
2.6.1.51
6.3.2.6
N
Xanthine
1.1.3.22
O-
ATP
HOCH2CH(NH2)COO-
2.7.1.28
P
OH OH
1.3.1.35
Stearoyl-CoA
Betaine
aldehyde
NADH
H
H
HN
OC
3.6.1.3
2.7.4.6
FOLIC
ACID
C1
POOL
2.1.2.1
+
OHCCH2N(CH3)3
6.3.4.3
6.3.4.17
RP
O
C
NH2
TTP
1.2.1.8
1.1.99.1
N
PURINE & PYRIMIDINE
METABOLISM
Benzoyl-CoA
COO
Hydroxybenzoate
-OOC.CH.CH COO2
N
HNCOC
CH
H2N C
N RP
NH2 N
C
C
N
N
N
CH2 O
O
Coenzyme-Q
CH
2.7.4.4
OH
Cyclic AMP
Betaine
1.2.1.12
NADPH
P
O-
O-
4.6.1.1
O
O
C
1.1.1.204
d-ADP
1.2.1.13
PC
O
~O P ~O
Phenyl acetate
-
Hydroxyphenyl
acetate
5-Amino-4-imidazole
5-Amino-4-imidazole
5-Aminoimidazole
carboxylate-RP (N-succinylcarboxamide)-RP carboxamide-RP
NH
6.2.1.25
3.5.1.32
OH
Hydroxymandelate
N
H2N
CO
C
N
H
COSCoA
1.2.1.39
OH
OH
N
C
+
-OOCCH N (CH )
2
3 3
NAD+
Pi
P
4.1.1.21
Benzoate
1.14.13.12
1.2.1.3
N
N
O
1.2.1.7
OH
URATE
NH2
-O
O
C
OC
1.7.3.3
Allantoin
N
O
1.4.4.2
2.1.2.10
CO
NH
N
N
CH2 O
O
I
Non-ketotic hyperglycinaemia
Glycogen Cleavage System
Glycine dehydrogenase (decarboxylating) 1.4.4.2
Aminomethyltransferase 2.1.2.10
COO-
CH2COO-
1.2.1.3
Hydroxyphenyl
glycolaldehyde
O
RP
Benzaldehyde
Phenyl
acetaldehyde
CH2COO-
1.4.3.4
C
2.1.1.5
2.4.2.14
6.3.4.7
Pi
.1
P
Dimethylglycine
OCH2CHOHCHO
3-P-Glyceraldehyde
OH OH
O
-O
1.5.99.2
-OOCCH N(CH )
2
3 2
C
N H
H
3.5.2.5
Sarcosine
OH OH
P-Ribosyl
amine
P
NH2
N
-OOCCH NHCH
2
3
Sedoheptulose-P
OC
NH
Hydroxyphenylacetaldehyde
CH(OH)CHO
C
HN
Phenyl
ethylamine
CH2CHO
OH
-OOC
NH
OC
Allantoate
1.4.1.10
2.6.1.4 1.5..3.1
1.5.99.1
2.1.1.20
NH2
RP 6.3.3.1
Hypersarcosinaemia
Sarcosine dehydrogenase 1.5.99.1
CHO
OH
OH
Hydroxyphenylethanolamine
CH
N
CH2CH2NH2
1.4.3.4
MELANIN THYROXINE
C
Tyrosine-negative Albinism
Monophenol monooxygenase 1.14.18.1
Phenyl
pyruvate
CH2CHO
CH(OH)COO-
HC
2.6.1.5
PHENYLALANINE
1.4.3.4
CH(OH)CH2NH2
I
N
H
O
CO
C
N H
H
OC
GLYCINE
O
I
I
H2N
1.14.16.1
Tyramine
O
NH2
COO-
NH2
3.5.3.4
P
O
N
6.3.5.3
H2NCONH2
4.1.2.13
COO-
CO
H
N
H2N
Dopamine monooxygenase 1.14.17.1
CH2CO COO-
1.14.17.1
+
CH2CH(NH3)COO-
GlycinamideFormyl
Formyl
5-Aminoimidazole-RP
ribosyl-P glycinamide-RP glycinamidine-RP
H
OH OH
6.3.4.13
P
OH OH OH H
OH
CHO
4.2.1.51
+
OH
Normetadrenaline
C
NH
RP
2.1.2.2
CH2O
P
Oleoyl-CoA
HN
NH
OH
Prephenate
CH2CH(NH3)COO-
4.1.1.53
1.14.18.1
NH
H2C
CHO
OC
1.13.11.1
CH2CH2NH2
Dopaquinone
OCH3
4-Hydroxy-3-methoxy
phenylglycol
(MOPEG)
H2C
NHCOCH2NH2
P
4.1.2.9
2-Oxo-3-deoxy6-P Gluconate
Plasto
cyanin P700
Photosystem
I
ACYL-ACP
C
Fructose1:6-bis-P
2.2.1.2
D-Ribose-5-P
2e-
1.10.99.1
2.1.1.6
P
O
OH OH H
CO
CHO
OH OH OH
COO-
Fructose-1,6-bis-phosphate aldolase 4.1.2.13
3, 4-Dihydroxy
phenylglycol
Phenylketonuria
Phenylalanine 4-monooxygenase 1.14.16.1
CH2COCOO-
4.1.1.25
OH
O
NH
CH2O
HO
H
D-RIBOSE
b6
Cytochrome
complex
f
Plasto
CH3(CH2)n+2CO.S-ACP
C
CHOHCH2NH2
OH
OH
OH
5.4.99.5
TYROSINE
2.6.1.5
Dopa
OCH3
ADP
COO-
6-Phosphofructokinase 2.7.1.11
CH(OH)CH2OH
1.14.12.1
+
OH
H2
C
CH.COO 1.14.18.1
+
NH3
O
OCH3
CH2OH
2.7.1.11
H
Dopamine
CH(OH)CH2OH
OH OH
D-Xylulose-5-P
HO
Glucose-6-phosphate isomerase 5.3.1.9
CO
Catechol
1.13.11.2
OH
4.1.1.28
O
4-Hydroxy-3-methoxyD-mandelate
(VMA)
2-Amino
muconate
COO-
Hereditary Tyrosinaemia (Type II)
Tyrosine aminotransferase 2.6.1.5
NH2
-OOC
Cis,cis-muconate
CH2CH(NH2)COO1.14.16.2
OH
OH
3, 4-DihydroxyD-mandelate
Hawkinsuria
4-Hydroxyphenylpyruvate dioxygenase 1.13.11.27
Chorismate
OH
CH2CH(NH3)COO-
Hydroxyphenyl
1.13.11.27
pyruvate
CH2CH2NH2
4
1.4.3.
4.1.3.27
OC — COO-
OH
4.6.1.4
Shikimate-5
enolpyruvate 3-P
-OOC
5.5.1.1
CH2
O — C — COO-
O
OH
2.5.1.19
P
OH
COOCOO-
OH
CH(OH)COO-
OH
OH
Alcaptonuria
Homogentisate 1,2-dioxygenase 1.13.11.5
1.3.1.13
OH
1.14.17.1
O
P
1.2.1.32
Anthranilate
COO-
COO-
Shikimate 2.7.1.71Shikimate-3-P
PEP
CH2COCOO-
Homogentisate
Maleyl
acetoacetate
Noradrenaline
OH
2.1.1.28
4
.3.
1.4
CH2(NH2)COOH
2e-
1.14.99.5
C
3.1.3.11
P
OCH2
P
CH2OH
Pyruvate carboxylase 6.4.1.1
Hexokinase 2.7.1.1
C
Erythrose-4-P
5.3.1.6
5.1.3.4
2.7.1.17
C
O
CH2COOCOOH
1.13.11.5
OH
2-Hydroxymuconate
semialdehyde
Muconolactone
CH2COO-
2.1.1.6
CHO
5.1.3.1
CH2OH
Ferredoxin
1.14.99.25
C
ATP
OH H
H
ADRENALINE
CH(OH)COO-
Fructose-6-P
D-Ribulose-5-P
ADP
Chlorophyll
Linoleate
HO
H
OCH2 C
P
2H+
Light-driven electron flow from H2O to NADP+
NON-SPHEROCYTIC
HAEMOLYTIC ANAEMIAS DUE TO
ERYTHROCYTE ENZYMOPATHIES
OH
5.3.3.4
OH
OH
OH
H
O
3-Oxoadipate
enol-lactone
OH
OH
H
C
C
H2
3.1.1.24
OH
5.3.1.9
H
C
CHOHCH2NHCH3
OH
OH OH
CO
C
H2
3-Oxoadipate
5.2.1.2
H2
C
COOHC
O
HC
C
C
O
H
COO-
O
HC
OH
HO OH
H2
C
C
CHOHCH2NH2
ADP
OH OH H
OH
C
Fumaryl
acetoacetate
D-Xylulose
Mn
O2
3.1.1.17
4.2.1.12
OH
OH H
P680
1/2
OH
2.8.3.6
O
CH2COO-
-OOC
3.7.1.2
CH2O P
O
NADPH
H
C
H2C
1.1.1.44
P
Fructose-1-P
2.7.1.47
CH2OH
ATP
quinone
3.1.3.9
COO-
OH
3.7.1.9
2-Oxopent-4-enoate
COOCOO-
O
ATP
HO OH
OH
H2
C
C
O
5.4.2.2
2.6.1.16
O
4
5.1.3.1
C
3-Oxoadipyl-CoA
2.7.1.2
2.7.1.1
2.7.1.
Dehydroshikimate
CH2=CH.CH2COCOO-
4.2.1.80
4-Hydroxy-2-oxovalerate
OH
UDP-Galactose
OH
2.4.2.18
N-(5-P-ribosyl) anthranilate
OH
1.1.1.25
CHO
COO-
OH
CH2O P
O
COO-
C
Dehydroquinate
4.6.1.3
O
HO OH
1.1.1.21
CH2OH
OH
NADP+
OH H
H
Ribitol
2e-
C
OH
CH3CH(OH)CH2COCOO-
2.2.1.1
L-Ribulose-5-P
2.7.1.16
PQ
Fructose-1,6-bis-phosphatase 3.1.3.11
Phosphoenolpyruvate carboxykinase (GTP) 4.1.1.32
H
C
Pheophytin
+
Glucose-6-phosphatase 3.1.3.9
H
OH
OH OH H
OH
P OCH2
C
OH H
OH OH OH
H2O
C
OH OH
PhotoSystem
II
Chlorophyll
–
REDOX POTENTIAL
Phosphogluconate dehydrogenase (decarboxylating) 1.1.1.44
OH H
C
6-P-Gluconate
PHOTOSYNTHESIS
PENTOSE PHOSPHATE PATHWAY
H
OH OH H
2.7.1.3
D-Xylose
CO
H
HOCH2
CH2OH
C
C
2.7.1.47
1.1.1.9
CH2OH
H
HOCH2 C
L-Xylulose-5-P
5.3.1.15
D-Arabitol
OCH2
O
HOCH2 C
CO
H
C
OH H
CHO
Fructose intolerance
Fructose-1.6-bis-phosphate aldolase (isoenzyme B) 4.1.2.13
C
OPPU
GLUCOSE
Sorbitol
1.10.2.1
1.10.3.3
3-Deoxy-D-arabinoheptulosonic acid 7-P
OH
CH2OH
O
NH2
3.2.1.48
3.2.1.26
SUCROSE
1.1.1.14
P
H
COONH2
P
OH OH
O
CH2
CoA.S.COCH2COCH2CH2COO-
D-RIBULOSE
OH H
C
OH OH
C
H
2.3.1.4
2.4.1.13
CH2OH
OH OH
L-RIBULOSE
OH OH
OH H
HOCH2
CHO
Glucosamine-6-P
CH2O P
O
P
4.1.1.48
HO
OH
4.2.1.10
Hereditary Tyrosinaemia (Type I)
Fumarylacetoacetase 3.7.1.2
N-Acetyl-5-O-methyl-Serotonin
(MELATONIN)
OH
O
OH
O
5.1.3.2
Glucose-1-P
HO OH
CO
5.5.1.4
Dehydroascorbate
OH OH
CHO
H
C
2.7.7.24
COO-
HO
CH2
2
Hyperserotoninaemia
Aromatic amino acid decarboxylase 4.1.1.28
CH2CH2NHCOCH3
H
H
COONH — CH — C — C — CH— CH2O
1-(o-Carboxy phenylamino)
COOCOO1-deoxyribulose-5-P
HO
OH
2.4.1.9
Indole-3-glycerol-P
HOCH HCOH
C
OH
OH
TRYPTOPHAN
NH2
COO-
NH
N-Acetyl-Serotonin 2.1.1.4
CH
N
H
Tryptophanuria
Tryptophan 2,3-dioxygenase 1.13.11.11
2-Aminomuconate6-semialdehyde
4.1.1.45
CH3O
NH
2.3.1.5
HOC—CH(OH)CH(OH)CH2O
P
CH
OCH
OC
P OCH
CH2OH
O
2.7.7.12
C—CH(OH)CH(OH)CH2O
N
H
4.2.1.20
OP
HO OH
OH H
C
HOCH2
5.3.1.4
H
C
HOCH2
Xylitol
H
C
H
COO-
CO
CH2O P
O
2.4.1.22
OP
Galactose--P
2.7.7.9
2.7.7.34
5.3.1.8
CO
H
H
1.1.1.10
D-LYXOSE
Fructokinase deficiency 2.7.1.4
CO
C
H
L-LYXOSE
Essential fructosuria
Ketohexokinase 2.7.1.3
OH
C
L-Arabinose
FRUCTOSE
C
H
L-Xylulose
H
OH H
C
OPPT
OH
HO OH
OH OH H
OH H
CH2OH
C
C
OH H
H
OH OH H
HOCH2
OH
C
2.7.1.6
CH2CH(N H3)COO-
NH
1.4.1
.19
5-Hydroxytryptamine
(SEROTONIN) COO-
Quinolinate
NH2
COO-
CH2CH2NHCOCH3
HO
NH
4.1.1.28
OCH
3-Hydroxy 1.13.11.6 2-Amino-3-carboxy
anthranilate
muconate semialdehyde
CH2CH2NH2
HO
NH
5-Hydroxytryptophan
4
1.14.16.
+
COO-
OH
CH2OH
O
TDP-Glucose
Mannose-1-P
O
H
D-ARABINOSE
L-Arabitol
Pentosuria
L-Xylulose reductase 1.1.1.10
C
H
OH OH H
4.1.1.34
C
C
H
CHO
OH H
H
C
OH OH H
ASCORBATE
2, 3-Dioxogulonate
L-XYLOSE
Sorbitol dehydrogenase deficiency 1.1.1.14
H
HOCH2 C
HO
GDP-Glucose
OH
O
3-Dehydrogulonate
Galactose-1-phosphate uridylyltransferase 2.7.7.12
OP
HO OH
C
HOCH2
CO
2-Oxogulonolactone
1.1.3.8
OH
H
OH
3.2.1.20
4.1.99.1
Indolepyruvate
CH2OH
O
OH
HO OH
HO OH HO O P
OH
Inositol OH
3.1.3.25
Inositol-P
OH OH
OH H
H
O
b-Galactosidase 3.2.1.23
OH
HO OH
OH
1.1.1.19
H
OH
H
Gulonate
GALACTOSE
OH
OH
C COO-
CH2OH
O
N-Ac-glucosamine-6-P NHCOCH3
5.4.2.3
1.1.1.22
4.1.1.28
NH
OH
GALACTOSE
UDP-Glucose
4.2.1.46
2.7.7.13
N-Ac-glucosamine-1-P
2.7.7.23
OPPU
2.7.7.27
CH2OH
O
5.4.2.8
CH2OH
O
GDP-Mannose
OP
TDP-4-Oxo6-deoxyglucose
Mannose-6-P
OH HO OPPG
NHCOCH3
UDP-N-Acetyl
glucosamine
O
MONO- and DI-SACCHARIDE
METABOLISM
HO OH
OPPU
NHAC
HO OH
2.4.1.1
OH
4.2.1.47
HO OH HO OPPG
CH2OH
O
5.1.3.13
OPPT
OH
NH
Tryptamine
CH2COCOO-
OH
OH
ADP-Glucose
OH
O
UDP-Glucuronate
CH2OH
O
O
1.13.11.11
+
N
2.4.2.19
Quinolinate-RP
COO-
OH
3.7.1.3
Xanthurenic aciduria
Kynureninase 3.7.1.3
COOCOO-
RP
2.4.2.19
NH2
NH2
CH2CH(N H3)COO-
HO
RP
COO-
OH
3-Hydroxy
kynurenine
COOCOO-
+
N
Nicotinic-RP
COCH2CH(N H3)COO-
1.14.13.9
Kynurenine
+
N
2.4.2.
11
2.7.7.18
+
NH2
CH2CH2NH2
4.1.1.43
COO-
N
NICOTINATE
O-
Desamino-NAD
6.3.1.5
COCH2CH(N H3)COO-
Formyl 3.5.1.9
kynurenine
NH
Indole
6.3.5.1
+
CHO
Indoleacetaldehyde
O
O
O-
+
N
H
NH
COO-
R — O — P — O — P — O — Adenosine
NAD
COCH2CH(N H3)COO-
CH2OH
O
3.2.1.23
HO
CH2OH
O
OH
2.7.1.23
NADP
CH2CHO
OH
OH
O
O
HO OH HO OH
OPPU
OH
N-Ac-Mannosamine-6-P
II a-1,4-Glucosidase 3.2.1.20 (Pompe)
2.7.1.38
CH3
OH
+
NH
Indoxyl
1.2.3.7
LACTOSE
2.4.1.21
TDP-Rhamnose
CH2O P
O
GDP-4-Dehydro6-deoxymannose
O
UDP-N-Ac-Glucosamine
3.1.3.29
pyruvate
4.1.3.20
CH3
UDPGalacturonate
COO-
NHAC
COO-
ACNH
HO OH
OH
V Muscle phosphorylase 2.4.1.1 (McArdle)
OPPU
CH2 C
CH2OH P
O
OPPU
OH
UDP-N-AcGalactosamine
2.4.1.18
2.4.1.29
+
N
O
+
OH
OH
OH
OH
5.1.3.12
NHCOCH3
OH
3.2.1.33
OPPT
OH OH
2.7.1.7
GDP-4-Oxofucose
OPPU
OH
CH2OH
O
HO
O
HO
CH2OH
O
O
MANNOSE
HO OPPG
COO-
HO
2.4.1.11
HO OH HO OH
2.4.1.32
CH3
O
CH2OH
O
1.1.1.158
OH
O
UDPIduronate
NHAC
O
Indoleacetate
(Auxin)
CH2OH
O
O
HO CH3
GDP-Fucose
OH
GLYCOGEN
CELLULOSE
CH2OH
O
HO OPPG
HO
2.4.1.68
2.4.1.69
OPPU
OPPU
HO O
VIII Liver phosphorylase b kinase 2.7.1.38 (Hug)
INULIN
STARCH
O
R — O — P — O — P — O— Adenosine(P)
OH
NH
HO
CH3
O
COO-
2.4.1.17
HO OH
IV 1,4-Glucan branching 2.4.1.18 (Andersen)
O-ANTIGENS
O
CH2OH
O
6.3.2.7-10
6.3.2.13
OPC
+
N
CH2COO-
PEPTIDO
GLYCAN
I Glucose-6-phosphatase 3.1.3.9 (von Gierke)
COO-
CONH2
Glycine
3.5.2.10
Electron Flow from Reduced NAD and Succinate to Oxygen
Biosynthesis
Degradation
Amino Acids Biosynthesis
Degradation
Hyperammonaemia (Type II)
Or nithine carbamoyltransferase 2.1.3.3
Most characteristics of human
anatomy and physiology are
determined by biochemical reactions
Degradation
Lipids Biosynthesis
catalysed by thousands of specific
Purines & Pyrimidines Biosynthesis
Degradation
enzymes. These, in turn, are
determined by our genetic make-up,
Pentose Phosphate Pathway
Photosynthesis
and genes are inherited – or inborn. If
Human Metabolism Vitamins, Co-enzymes, Hormones
a gene is defective or missing it may
result in defective or missing enzyme.
Small numbers refer to Enzyme Commission
Reference Numbers for Enzymes
This may well be lethal, but in some
cases it can lead to a Metabolic
Deficiency Disease or Inborn Error of
Metabolism. For example, in the metabolic pathway a
b
c
d, if the enzyme which
catalyses the reaction b
c is defective, c may not be formed. The reaction sequence may,
however, continue to produce b which can then concentrate in body fluids such as blood or
urine. The knowledge of the site of the defective enzyme and of its corresponding gene(s) may
well make possible the rational treatment of the resulting disease. The Inborn Errors of
Metabolism Map names about 120 such diseases and identifies their sites of origin.
Biochemical understanding thus provides an insight into clinical understanding.
Hyperammonaemia (Type I)
Carbamoylphosphate synthase (ammonia) 6.3.4.16
Argininosuccinic aciduria
Argininosuccinate lyase 4.3.2.1
Hyperornithinaemia (Type I)
Ornithine-oxoacid aminotransferase 2.6.1.13
Hyperargininaemia
Arginase 3.5.3.1
Product No. I 8014
© 1998 20th Edition Designed by Dr. Donald E. Nicholson, Department of Biochemistry and Molecular Biology, The University of Leeds, England – and Sigma
Product No. I 8014
Biochemicals and Reagents
for Life Science Research
Argentina
SIGMA-ALDRICH
de ARGENTINA, S.A.
Tel: 54 1 807-0321
Fax: 54 1 807-0346
Australia
SIGMA-ALDRICH PTY., LIMITED
Free Tel: 1-800-800-097
Free Fax: 1-800-800-096
Sydney Tel: 61-2-9841-0555
Sydney Fax: 61-2-9841-0500
Canada
SIGMA-ALDRICH CANADA LTD.
Free Tel: 800-565-1400
Free Fax: 800-265-3858
Tel: 905-829-9500
Fax: 905-829-9292
Czech Republic
SIGMA-ALDRICH s.r.o.
Tel: 02-2317 361
Fax: 02-2317 356
Austria
SIGMA-ALDRICH Handels GmbH
Tel: 01-605-81-10
Fax: 01-605-81-20
Finland
Sigma-Aldrich Finland
YA-Kemia Oy
Tel: 358-9-350-9250
Fax: 358-9-350-92555
Belgium
SIGMA-ALDRICH NV/SA
Free Tel: 0800-14747
Free Fax: 0800-14745
Tel: 03 899 13 01
Fax: 03 899 13 11
France
SIGMA-ALDRICH CHIMIE S.à.r.l.
Tél Numéro Vert: 0800 21 14 08
Fax Numéro Vert: 0800 03 10 52
Téléphone: 04 74 82 29 20
Télécopie: 04 74 95 68 08
Brazil
SIGMA-ALDRICH QUÍMICA
BRASIL LTDA.
Tel: (011) 231 1866
Fax: (011) 257 9079
Germany
SIGMA-ALDRICH CHEMIE GmbH
Service Tel: 0130 5155
Service Fax: 0130 6490
Telefon: 089/6513-0
Telefax: 089/6513-1169
Fax from Africa & Middle East
49 (089) 6513 1888
Fax from CIS, Baltics & SE Europe
49 (089) 6513 1877
Greece
Sigma-Aldrich (O.M.) Ltd.
Tel: 01 9943830
Fax: 01 9943831
Hungary
SIGMA-ALDRICH Kft
Tel: (06-1) 269-6474
Fax: (06-1) 235-9050
Ingyenes zöld telefon:
06-80-355-355
Ingyenes zöld fax:
06-80-344-344
India
SIGMA-ALDRICH CORP.
Hyderabad Location
Tel: (040) 244 739
Fax: (040) 244 794
New Delhi Location
Tel: (011) 689 9826
Tel: (011) 689 7830
Fax: (011) 689 9827
Ireland
SIGMA-ALDRICH IRELAND LTD.
Free Tel: 1-800-200-888
Free Fax: 1-800-600-222
Tel: 01-404-1900
Fax: 01-404-1910
Israel
SIGMA ISRAEL CHEMICALS LTD.
Free Tel: 1-800-70-2222
Tel: (08) 9484-222
Fax: (08) 9484-200
Italy
SIGMA-ALDRICH S.r.l.
Numero Verde: 167-827018
Telefono: (02) 33417-310
Fax: (02) 38010737
Japan
SIGMA-ALDRICH JAPAN K.K.
Toll-Free Tel: 0120-07-0406
Toll-Free Fax: 0120-67-6788
Tokyo Tel: (03) 5640 8885
Tokyo Fax: (03) 5640 8857
Korea
SIGMA-ALDRICH KOREA
Toll Free Tel: 080-023-7111
Toll Free Fax: 080-023-8111
Tel: 02-783-5211
Fax: 02-783-5011
Mexico
SIGMA-ALDRICH QUÍMICA
S.A. de C.V.
Free Tel: 01-800-007-5300
Tel: 01-5-631-3671 (Mexico City)
Fax: 01-5-631-3780
The Netherlands
SIGMA-ALDRICH CHEMIE BV
Tel Gratis: 0800-022 90 88
Fax Gratis: 0800-022 90 89
Tel: 078-6205411
Fax: 078-6205421
Norway
SIGMA-ALDRICH NORWAY AS
Tel: 22 09 15 00
Fax: 22 09 15 10
Poland
SIGMA-ALDRICH Sp. z o.o
Tel: (061) 823-2481
Fax: (061) 823-2781
Portugal
SIGMA-ALDRICH QUIMICA, S.A.
Free Tel: 0800.20.21.78
Free Tax: 0800.20.21.78
Tel: 351-1-9242555
Fax: 351-1-9242610
Russia
SIGMA-ALDRICH RUSSIA
Techcare Systems, Inc.
(TechMedBioChem)
Tel: 7 095 975 3321
Fax: 7 095 975 4792
Singapore
SIGMA-ALDRICH PTE., LTD.
Tel: 65 773 6028
Fax: 65 773 6086
South Africa
SIGMA-ALDRICH S. A. (PTY.) LTD.
Toll Free Tel: 0800 110075
Toll Free Fax: 0800 110079
Tel: 011-805-5230
Fax: 011-805-5215
United Kingdom
SIGMA-ALDRICH COMPANY LTD.
Free Tel: 0800 717181
Free Fax: 0800 378785
Tel: 01202 733114
Fax: 01202 715460
Spain
SIGMA-ALDRICH QUÍMICA S.A.
Free Tel: 900-10-1376
Free Fax: 900-10-2028
Tel: 34-1-6619977
Fax: 34-1-6619642
United States
SIGMA
P.O. Box 14508
St. Louis, Missouri 63178-9916
Toll Free: 1-800-521-8956
Call Collect: 314-771-5750
Toll-Free Fax: 800-325-5052
Tel: 314-771-5750
Fax: 314-771-5757
All other calls: 314-771-5765
Sweden
SIGMA-ALDRICH SWEDEN AB
Tel: 020-35 05 10
Fax: 020-35 25 22
Outside Sweden Tel: 08-7424200
Outside Sweden Fax: 08-7427299
Switzerland
SIGMA CHEMIE
Swiss Free Call: 0800 80 00 80
Tel: 081 755 2721
Tel: 081 755 2723
Fax: 081 755 2840
Internet
http://www.sigma.sial.com
Quantity Pricing / Standing Orders:
800-521-8956 or 314-771-5765 Ext. 3702
Development / Manufacturing Inquiries:
800-336-9719 • 314-534-4900
OXIDOREDUCTASES
1.14.12.10 Benzoate 1,2-dioxygenase
2.7.2.3
Phosphoglycerate kinase
4.1.1.49
Phosphoenolpyruvate carboxykinase (ATP)
1.14.13.5
Imidazoleacetate 4-monooxygenase
2.7.2.4
Aspartate kinase
4.1.1.50
Adenosylmethionine decarboxylase
1.14.13.9
Kynurenine 3-monooxygenase
2.7.2.6
Formate kinase
4.1.1.53
Phenylalanine decarboxylase
1.14.13.12
Benzoate 4-monooxygenase
2.7.2.11
Glutamate 5-kinase
4.1.1.65
Phosphatidylserine decarboxylase
1.14.13.39
Nitric oxide synthase
2.7.3.2
Creatine kinase
4.1.1.71
2-Oxoglutarate decarboxylase
1.14.16.1
Phenylalanine 4-monooxygenase
2.7.4.2
Phosphomevalonate kinase
4.1.2.5
Threonine aldolase
1.14.16.2
Tyrosine 3-monooxygenase
2.7.4.3
Adenylate kinase
4.1.2.9
Phosphoketolase
1.14.16.4
Tryptophan 5-monooxygenase
2.7.4.4
Nucleoside-phosphate kinase
4.1.2.12
Ketopantoaldolase
1.14.17.1
Dopamine ß-monooxygenase
2.7.4.6
Nucleoside-diphosphate kinase
4.1.2.13
Fructose-bisphosphate aldolase
1.14.18.1
Monophenol monooxygenase
2.7.4.9
dTMP kinase
4.1.2.14
2-Dehydro-3-deoxyphosphogluconate aldolase
1 14.99.1
Prostaglandin synthase
2.7.4.14
Cytidylate kinase
4.1.2.15
2-Dehydro-3-deoxyphosphoheptonate aldolase
1 14.99.5
Stearoyl-CoA desaturase
2.7.6.1
Ribose-phosphate pyrophosphokinase
4.1.3.1
Isocitrate lyase
1.14.99.7
Squalene monooxygenase
2.7.7.3
Pantetheine-phosphate adenylyltransferase
4.1.3.2
Malate synthase
1.14.99.25
Linoleoyl-CoA desaturase
2.7.7.4
Sulfate adenylyltransferase
4.1.3.4
Hydroxymethylglutaryl-CoA lyase
1.17.4.1
Ribonucleoside-diphosphate reductase
2.7.7.6
RNA nucleotidyltransferase (DNA-directed)
4.1.3.5
Hydroxymethylglutaryl-CoA synthase
1.18.6.1
Nitrogenase
2.7.7.7
DNA nucleotidyltransferase (DNA-directed)
4.1.3.7
Citrate (si)-synthase
2.7.7.9
UTP—glucose-1-phosphate uridylyltransferase
4.1.3.8
ATP citrate (pro-S)-lyase
2.7.7.10
UTP—hexose -1-phosphate uridylyltransferase
4.1.3.16
4-Hydroxy-2-oxoglutarate aldolase
Nicotinamide N-methyltransferase
2.7.7.12
UDP glucose—hexose-1-phosphate uridylyltransferase
4.1.3.18
Acetolactate synthase
2.1.1.2
Guanidinoacetate N-methyltransferase
2.7.7.13
Mannose-1-phosphate guanylyltransferase
4.1.3.20
N-Acylneuraminate-9-phosphate synthase
2.1.1.3
Thetin—homocysteine S-methyltransferase
2.7.7.14
Ethanolamine-phosphate cytidylyltransferase
4.1.3.21
Homocitrate synthase
Acetylserotonin N-methyltransferase
2.7.7.15
Choline-phosphate cytidylyltransferase
4.1.3.22
Citramalate lyase
Betaine—homocysteine S-methyltransferase
2.7.7.18
Nicotinate-mononucleotide adenylyltransferase
4.1.3.27
Anthranilate synthase
Catechol O-methyl transferase
2.7.7.22
Mannose-1-phosphate guanylyltransferase (GDP)
4.1.99.1
Tryptophanase
2.1.1.10
Homocysteine S-methyltransferase
2.7.7.23
UDP-N-acetylglucosamine pyrophosphorylase
4.2.1.2
Fumarate hydratase
2.1.1.13
5-Methyltetrahydrofolate—homocysteine
S-methyl transferase
2.7.7.24
Glucose-1-phosphate thymidylyltransferase
4.2.1.3
Aconitate hydratase
2.7.7.27
Glucose-1-phosphate adenylyltransferase
4.2.1.4
Citrate dehydratase
5-Methyltetrahydropteroyltriglutamate —
homocysteine S-methyltransferase
2.7.7.34
Glucose-1-phosphate guanylyltransferase
4.2.1.9
Dihydroxy-acid dehydratase
2.7.7.41
Phosphatidate cytidylyltransferase
4.2.1.10
3-Dehydroxyquinate dehydratase
1.1.1.1
Alcohol dehydrogenase
1.1.1.8
Glycerol-3-phosphate dehydrogenase
1.1.1.9
D-Xylulose
reductase
1.1.1.10
L-Xylulose
reductase
1.1.1.14
L-Iditol
dehydrogenase
1.1.1.15
D-Iditol
dehydrogenase
1.1.1.19
Glucuronate reductase
1.1.1.21
Aldehyde reductase
1.1.1.22
UDPglucose dehydrogenase
1.1.1.23
Histidinol dehydrogenase
1.1.1.25
Shikimate dehydrogenase
1.1.1.27
Lactate dehydrogenase
1.1.1.28
D-Lactate
1.1.1.29
Glycerate dehydrogenase
1.1.1.30
3-Hydroxybutyrate dehydrogenase
1.1.1.31
3-Hydroxyisobutyrate dehydrogenase
1.1.1.32
Mevaldate reductase
1.1.1.34
Hydroxymethylglutaryl-CoA reductase (NADPH)
1.1.1.35
3-Hydroxyacyl-CoA dehydrogenase
1.1.1.37
Malate dehydrogenase
1.1.1.38
Malate dehydrogenase (oxaloacetate-decarboxylating)
1.1.1.39
Malate dehydrogenase (decarboxylating)
1.1.1.41
Isocitrate dehydrogenase (NAD+)
1.1.1.43
Phosphogluconate dehydrogenase
1.1.1.44
Phosphogluconate dehydrogenase (decarboxylating)
1.1.1.45
L-Gulonate
1.1.1.49
Glucose-6-phosphate dehydrogenase
1.1.1.56
Ribitol dehydrogenase
2.1.1.17
Phosphatidylethanolamine N-methyltransferase
2.7.7.43
N-Acylneuraminate cytidylyltransferase
4.2.1.11
Phosphopyruvate hydratase (enolase)
1.1.1.59
3-Hydroxypropionate dehydrogenase
2.1.1.20
Glycine N-methyltransferase
2.7.8.1
Ethanolamine phosphotransferase
4.2.1.12
Phosphogluconate dehydratase
1.1.1.60
2-Hydroxy-3-oxopropionate reductase
2.1.1.28
Phenylethanolamine N-methyltransferase
2.7.8.2
Diacylglycerol cholinephosphotransferase
4.2.1.13
L-Serine
1.1.1.61
4-Hydroxybutyrate dehydrogenase
2.1.1.43
Histone-lysine N-methyltransferase
Ceramide cholinephosphotransferase
4.2.1.16
Threonine dehydratase
1.1.1.79
Glyoxylate reductase (NADP+)
2.7.8.3
2.1.1.45
Thymidylate synthase
2.7.8.5
CDPdiacylglycerol—glycerol-3-phosphate 3-phosphatidyltransferase
4.2.1.17
Enoyl-CoA hydratase
1.1.1.81
Hydroxypyruvate reductase
2.1.2.1
Glycine hydroxymethyltransferase
CDPdiacylglycerol—serine O-phosphatidyltransferase
4.2.1.18
Methylglutaconyl-CoA hydratase
1.1.1.82
Malate dehydrogenase (NADP+)
2.7.8.8
2.1.2.2
Phosphoribosylglycinamide formyltransferase
2.7.8.11
CDPdiacylglycerol—inositol 3-phosphatidyltransferase
4.2.1.19
Imidazoleglycerol-phosphate dehydratase
1.1.1.85
3-Isopropylmalate dehydrogenase
2.1.2.3
Phospho-N-acetylmuramoyl-pentapeptide-transferase
4.2.1.20
Tryptophan synthase
Ketol-acid reductoisomerase
Phosphoribosylaminoimidazole carboxamide
formyltransferase
2.7.8.13
1.1.1.86
2.8.3.1
Propionate CoA-transferase
4.2.1.22
Cystathionine ß-synthase
1.1.1.95
Phosphoglycerate dehydrogenase
2.1.2.5
Glutamate formiminotransferase
2.8.3.5
3-Oxoacid CoA-transferase
4.2.1.24
Porphobilinogen synthase
1.1.1.100
3-Oxoacyl-[acyl-carrier-protein] reductase
2.1.2.10
Aminomethyl transferase
2.8.3.6
3-Oxoadipate CoA-transferase
4.2.1.33
3-Isopropylmalate dehydratase
1.1.1.102
3-Dehydrosphinganine reductase
2.1.3.1
Methylmalonyl-CoA carboxyltransferase
4.2.1.34
(S)-2-Methylmalate hydratase
1.1.1.105
Retinol dehydrogenase
2.1.3.2
Aspartate carbamoyltransferase
4.2.1.36
Homoaconitate hydratase
1.1.1.155
Homoisocitrate dehydrogenase
2.1.3.3
Ornithine carbamoyltransferase
1.1.1.157
3-Hydroxybutyryl-CoA dehydrogenase
2.1.4.1
Glycine amidinotransferase
1.1.1.158
UDP-N-acetylmuramate dehydrogenase
2.2.1.1
Transketolase
1.1.1.169
2-Dehydropantoate 2-reductase
2.2.1.2
Transaldolase
1.1.1.204
Xanthine dehydrogenase
2.3.1.1
Amino-acid N-acetyltransferase
1.1.1.205
IMP dehydrogenase
2.3.1.4
Glucosamine-phosphate N-acetyltransferase
1.1.3.8
L-Gulonolactone
2.3.1.5
Arylamine N-acetyltransferase
1.1.3.15
(S)-2-Hydroxyacid oxidase
2.3.1.6
Choline O-acetyltransferase
1.1.3.22
Xanthine oxidase
2.3.1.7
Carnitine O-acetyltransferase
1.1.99.1
Choline dehydrogenase
2.3.1.8
Phosphate acetyltransferase
1.1.99.5
Glycerol-3-phosphate dehydrogenase
2.3.1.9
Acetyl-CoA C-acetyltransferase
1.2.1.3
Aldehyde dehydrogenase (NAD+)
2.3.1.12
Dihydrolipoamide S-acetyltransferase
1.2.1.7
Benzaldehyde dehydrogenase (NADP+)
2.3.1.15
Glycerol-3-phosphate O-acyltransferase
1.2.1.8
Betaine-aldehyde dehydrogenase
2.3.1.16
Acetyl-CoA C-acyltransferase
1.2.1.11
Aspartate-semialdehyde dehydrogenase
2.3.1.20
Diacylglycerol O-acyltransferase
1.2.1.12
Glyceraldehyde-3-phosphate dehydrogenase
2.3.1.21
Carnitine O-palmitoyltransferase
1.2.1.13
Glyceraldehyde-3-phosphate dehydrogenase
2.3.1.23
Lysolecithin acyltransferase
1.2.1.16
Succinate-semialdehyde dehydrogenase (NAD(P)+)
2.3.1.24
Sphingosine N-acyltransferase
1.2.1.18
Malonate semialdehyde dehydrogenase (acetylating)
2.3.1.30
Serine O-acetyltransferase
1.2.1.19
Aminobutyraldehyde dehydrogenase
2.3.1.32
Lysine N-acetyltransferase
1.2.1.21
Glycolaldehyde dehydrogenase
2.3.1.35
Glutamate N-acetyltransferase
1.2.1.23
2-Oxoaldehyde dehydrogenase (NAD+)
2.3.1.37
5-Aminolevulinate synthase
1.2.1.24
Succinate-semialdehyde dehydrogenase
2.3.1.38
[Acyl-carrier-protein] S-acetyltransferase
1.2.1.25
2-Oxoisovalerate dehydrogenase (acylating)
2.3.1.39
[Acyl-carrier-protein] S-malonytransferase
1.2.1.26
2,5-Dioxovalerate dehydrogenase
2.3.1.41
3-Oxoacyl-[acyl-carrier-protein] synthase
1.2.1.27
Methylmalonate-semialdehyde dehydrogenase (acylating)
2.3.1.46
Homoserine O-succinyltransferase
1.2.1.28
Benzaldehyde dehydrogenase (NAD+)
2.3.1.50
Serine C-palmitoyltransferase
1.2.1.31
L-Aminoadipate-semialdehyde
2.3.1.51
1-Acylglycerol-3-phosphate O-acyltransferase
1.2.1.32
Aminomuconate-semialdehyde dehydrogenase
2.3.1.76
Retinol O-fatty-acyltransferase
1.2.1.36
Retinal dehydrogenase
2.4.1.1
Phosphorylase
1.2.1.39
Phenylacetaldehyde dehydrogenase
2.4.1.4
Amylosucrase
1.2.1.41
Glutamate-5-semialdehyde dehydrogenase
2.4.1.9
Inulosucrase
1.2.1.52
Oxoglutarate dehydrogenase
2 4.1.11
Glycogen (starch) synthase
1.2.3.5
Glyoxylate oxidase
2.4.1.13
Sucrose synthase
1.2.4.1
Pyruvate dehydrogenase (lipoamide)
2.4.1.17
Glucuronosyltransferase
1.2.4.2
Oxoglutarate dehydrogenase (lipoamide)
2.4.1.21
Starch synthase
1.2.7.1
Pyruvate synthase
2.4.1.22
Lactose synthase
1.2.7.2
2-Oxobutyrate synthase
2.4.1.23
Sphingosine ß-galactosyltransferase
1.3.1.1
Dihydrouracil dehydrogenase (NAD+)
2.4.1.29
Cellulose synthase (GDP-forming)
1.3.1.2
Dihydropyrimidine dehydrogenase (NADP+)
2.4.1.32
Glucomannan 4-ß-mannosyltransferase
1.3.1.6
Fumarate reductase (NADH)
2.4.1.41
Polypeptide N-acetylgalactosaminyltransferase
1.3.1.8
Acyl-CoA dehydrogenase (NADP+)
2.4.1.62
Ganglioside galactosyltransferase
1.3.1.9
Enoyl-[acyl-carrier-protein] reductase (NADH)
2.4.1.68
Glycoprotein 6-a-L-fucosyltransferase
1.3.1.10
Enoyl-[acyl-carrier-protein] reductase (NADPH, B-specific)
2.4.1.69
Galactoside 2-a-L-fucosyltransferase
1.3.1.13
Prephenate dehydrogenase (NADP+)
2.4.1.101
a-1,3-Mannosyl-glycoprotein ß-1,2-N-acetylglucosaminyltransferase
1.3.1.14
Orotate reductase (NADH)
2.4.2.1
Purine-nucleoside phosphorylase
1.3.1.26
Dihydrodipicolinate reductase
2.4.2.2
Pyrimidine-nucleoside phosphorylase
1.3.1.35
Phosphatidylcholine desaturase
2.4.2.4
Thymidine phosphorylase
1.3.3.3
Coproporphyrinogen oxidase
2.4.2.8
Hypoxanthine phosphoribosyltransferase
1.3.3.4
Protoporphyrinogen oxidase
2.4.2.9
Uracil phosphoribosyltransferase
1.3.5.1
Succinate dehydrogenase (ubiquinone)
2.4.2.10
Orotate phosphoribosyltransferase
1.3.99.1
Succinate dehydrogenase
2.4.2.11
Nicotinate phosphoribosyltransferase
1.3.99.2
Butyryl-CoA dehydrogenase
2.4.2.14
Amidophosphoribosyltransferase
1.3.99.3
Acyl-CoA dehydrogenase
2.4.2.15
Guanosine phosphorylase
1.3.99.7
Glutaryl-CoA dehydrogenase
2.4.2.17
ATP phosphoribosyltransferase
1.3.99.10
Isovaleryl-CoA dehydrogenase
2.4.2.18
Anthranilate phosphoribosyltransferase
1.4.1.1
Alanine dehydrogenase
2.4.2.19
Nicotinate-nucleotide pyrophosphorylase (carboxylating)
1.4.1.2
Glutamate dehydrogenase
2.4.99.7
Sialyltransferase
1.4.1.7
Serine dehydrogenase
2.4.99.1-11 Sialyl transferases
1.4.1.8
Valine dehydrogenase (NADP+)
2.5.1.1
Dimethylallyltransferase
1.4.1.9
Leucine dehydrogenase
2.5.1.6
Methionine adenosyltransferase
1.4.1.10
Glycine dehydrogenase
2.5.1.10
Geranyltranstransferase
1.4.1.13
Glutamate synthase (NADPH)
2.5.1.16
Aminopropyltransferase
1.4.1.14
Glutamate synthase (NADH)
2.5.1.19
3-Phosphoshikimate 1-carboxyvinyl-transferase
1.4.1.19
Tryptophan dehydrogenase
2.5.1.21
Farnesyltransferase
1.4.3.1
D-Aspartate
2.5.1.22
Spermine synthase
1.4.3.2
L-Amino-acid
2.5.1.29
Farnesyltranstransferase
1.4.3.4
Amine oxidase (flavin-containing)
2.5.1.32
Geranylgeranyl-diphosphate geranylgeranyl transferase
1.4.3.6
Amine oxidase (copper-containing)
2.6.1.1
Aspartate transaminase
1.4.3.8
Ethanolamine oxidase
2.6.1.2
Alanine transaminase
1.4.4.2
Glycine dehydrogenase (decarboxylating)
2.6.1.4
Glycine transaminase
2.6.1.5
Tyrosine transaminase
1.5.1.1
dehydrogenase
dehydrogenase
TRANSFERASES
2.1.1.1
2.1.1.4
2.1.1.5
2.1.1.6
2.1.1.14
oxidase
dehydrogenase
oxidase
oxidase
Pyrroline-2-carboxylate reductase
Pyrroline-5-carboxylate reductase
2.6.1.6
Leucine transaminase
1.5.1.3
Dihydrofolate reductase
2.6.1.9
Histidinol-phosphate transaminase
1.5.1.5
Methylenetetrahydrofolate reductase
2.6.1.11
Acetylornithine transaminase
1.5.1.6
Formyltetrahydrofolate dehydrogenase
2.6.1.13
Ornithine—oxo-acid transaminase
1.5.1.7
Saccharopine dehydrogenase (NAD+), L-lysine-forming
2.6.1.16
Glutamine—fructose-6-phosphate transaminase
1.5.1.8
Saccharopine dehydrogenase (NADP+), L-lysine-forming
2.6.1.17
Succinyldiaminopimelate transaminase
2.6.1.18
ß-Alanine—pyruvate transaminase
2.6.1.19
4-Aminobutyrate transaminase
2.6.1.22
L-3-Aminoisobutyrate
1.5.1.9
Saccharopine dehydrogenase
L-glutamate-forming+)
1.5.1.10
Saccharopine dehydrogenase (NADP,
1.5.1.12
1-Pyrroline-5-carboxylate dehydrogenase
1.5.1.21
∆ -Piperideine-2-carboxylate reductase
2.6.1.23
4-Hydroxyglutamate transaminase
1.5.3.1
Sarcosine oxidase
2.6.1.27
Tryptophan transaminase
2.6.1.32
Valine—3-methyl-2-oxovalerate transaminase
1.5.99.1
1
Sarcosine dehydrogenase
transaminase
1.5.99.2
Dimethylglycine dehydrogenase
2.6.1.36
L-Lysine
1.5.99.3
L-Pipecolate
2.6.1.39
2-Aminoadipate transaminase
1.5.99.8
Proline dehydrogenase
2.6.1.42
Branched-chain-amino-acid transaminase
1.6.4.1
Cystine reductase (NADH)
2.6.1.51
Serine—pyruvate transaminase
1.6.5.3
NADH dehydrogenase (ubiquinone)
2.6.1.52
Phosphoserine transaminase
1.6.6.1
Nitrate reductase (NADH)
2.6.1.65
b-lysine transaminase
N6-Acetyl-b
1.6.6.2
Nitrate reductase [NAD(P)H]
2.6.1.66
Valine—pyruvate transaminase
1.6.6.3
Nitrate reductase (NADPH)
2.7.1.1
Hexokinase
1.6.6.4
Nitrite reductase [NAD(P)H]
2.7.1.2
Glucokinase
1.6.6.8
GMP reductase
2.7.1.3
Ketohexokinase
1.7.3.3
Urate oxidase
2.7.1.4
Fructokinase
1.7.7.1
Ferredoxin—nitrate reductase
2.7.1.6
Galactokinase
dehydrogenase
6-transaminase
1.7.99.4
Nitrate reductase
2.7.1.7
Mannokinase
1.8.1.3
Hypotaurine dehydrogenase
2.7.1.11
6-Phosphofructokinase
1.8.1.4
Dihydrolipoamide dehydrogenase
2.7.1.15
Ribokinase
2.7.1.16
Ribulokinase
2.7.1.17
Xylulokinase
1.8.2.1
1.8.3.1
Sulfite dehydrogenase
Sulfite oxidase
1.8.7.1
Sulfite reductase (ferredoxin)
2.7.1.19
Phosphoribulokinase
1.8.99.1
Sulfite reductase
2.7.1.23
NAD+ kinase
1.9.3.1
Cytochrome-c oxidase
2.7.1.24
Dephospho-CoA kinase
1.10.2.1
L-Ascorbate—cytochrome-b5
2.7.1.25
Adenylylsulfate kinase
1.10.2.2
Ubiquinol—cytochrome-c reductase
2.7.1.28
Triokinase
reductase
oxidase
Triacylglycerol lipase
4.2 1.46
dTDPglucose 4,6-dehydratase
3.1.1.4
Phospholipase A2
4.2.1.47
GDPmannose 4,6-dehydratase
3.1.1.5
Lysophospholipase
4.2.1.49
Urocanate hydratase
3.1.1.7
Acetylcholinesterase
4.2.1.51
Prephenate dehydratase
3.1.1.17
Gluconolactonase
4.2.1.52
Dihydrodipicolinate synthase
3.1.1.21
Retinyl-palmitate esterase
4.2.1.55
3-Hydroxybutyryl-CoA dehydratase
3.1 1.24
3-Oxoadipate enol-lactonase
4.2.1.58
Crotonoyl-[acyl-carrier-protein] hydratase
3.1.1.28
Acylcarnitine hydrolase
4.2.1.59
3-Hydroxyoctanoyl-[acyl-carrier protein] dehydratase
3.1.1.31
6-Phosphogluconolactonase
4.2.1.60
3-Hydroxydecanoyl-[acyl-carrier protein] dehydratase
3.1.1.32
Phospholipase A1
4.2.1.61
3-Hydroxypalmitoyl-[acyl-carrier protein] dehydratase
3.1.2.1
Acetyl-CoA hydrolase
4.2.1.75
Uroporphyrinogen-III synthase
Succinyl-CoA hydrolase
4.2.1.80
2-Oxopent-4-enoate hydratase
3-Hydroxyisobutyryl-CoA hydrolase
4.2.99.2
Threonine synthase
Acetoacetyl-CoA hydrolase
4.2.99.8
Cysteine synthase
Acid phosphatase
4.2.99.9
O-Succinylhomoserine (thiol)-lyase
3.1.3.3
Phosphoserine phosphatase
4.3.1.1
Aspartate ammonia-lyase
3.1.3.4
Phosphatidate phosphatase
4.3.1.2
Methylaspartate ammonia-lyase
5´-Nucleotidase
4.3.1.3
Histidine ammonia-lyase
Glucose-6-phosphatase
4.3.1.4
Formiminotetrahydrofolate cyclodeaminase
Hydroxymethylbilane synthase
3.1.1.3
3.1.2.3
3.1.2.4
3.1.2.11
3.1.3.2
3.1.3.5
3.1.3.9
Fructose-bisphosphatase
4.3.I.8
3.1.3.15
Histidinol-phosphatase
4.3.2.1
Argininosuccinate lyase
3.1.3.25
myo-Inositol-1(or 4)-monophosphatase
4.3.2.2
Adenylosuccinate lyase
3.1.3.27
Phosphatidylglycerophosphatase
4.4.1.1
Cystathionine g-lyase
3.1.3.29
N-Acylneuraminate-9-phosphatase
4.4.1.8
Cystathionine ß-lyase
3.1.3.31
Nucleotidase
4.6.1.1
Adenylate cyclase
3.1.4.2
Glycerophosphocholine phosphodiesterase
4.6.1.3
3-Dehydroquinate synthase
3.1.4.3
Phospholipase C
4.6.1.4
Chorismate synthase
3.1.4.4
Phospholipase D
4.99.1.1
Ferrochelatase
3.1.4.10
1-Phosphatidylinositol phosphodiesterase
3.1.4.12
Sphingomyelin phosphodiesterase
3.1.3.11
1.10.3.3
L-Ascorbate
2.7.1.30
Glycerol kinase
1.10.99.1
Plastoquinol—plastocyanin reductase
2.7.1.31
Glycerate kinase
1.13.11.1
Catechol 1,2-dioxygenase
2.7.1.32
Choline kinase
1.13.11.2
Catechol 2,3-dioxygenase
2.7.1.33
Pantothenate kinase
1.13.11.5
Homogentisate 1,2-dioxygenase
2.7.1.34
Pantetheine kinase
1.13.11.6
3-Hydroxyanthranilate 3,4-dioxygenase
2.7.1.36
Mevalonate kinase
1.13.11.11 Tryptophan 2,3-dioxygenase
2.7.1.39
Homoserine kinase
1.13.11.20 Cysteine dioxygenase
2.7.1.40
Pyruvate kinase
1.13.11.21 ß-Carotene l5,15´-dioxygenase
2.7 1.47
D-Ribulokinase
1.13.11.27 4-Hydroxyphenylpyruvate dioxygenase
2.7.1.53
L-Xylulokinase
1.13.99.1
myo-lnositol oxygenase
2.7.1.60
N-Acylmannosamine kinase
1.13.99.2
Benzoate 1,2-dioxygenase
2.7.1.64
myo-Inositol 1-kinase
1.14.11.1
g-Butyrobetaine dioxygenase
2.7.1.71
Shikimate kinase
1.14.11.2
Procollagen-proline dioxygenase
2.7.1.80
Pyrophosphate—serine phosphotransferase
1.14.11.8
Trimethyllysine dioxygenase
2.7.1.107
Diacylglycerol kinase
1.14.12.1
Anthranilate 1,2-dioxygenase (deaminating, decarboxylating)
2.7.2.1
Acetate kinase
ISOMERASES
3.2.1.3
Glucan 1,4 -a-glucosidase
5.1.3.1
Ribulose-phosphate 3-epimerase
3.2.1.21
ß-Glucosidase
5.1.3.2
UDPglucose 4-epimerase
3.2.1.23
ß-Galactosidase
5.1.3.4
L-Ribulose-phosphate
3.2.1.26
ß-Fructofuranosidase
5.1.3.6
UDPglucuronate 4-epimerase
3.2.1.45
Glucosylceramidase
5.1.3.7
UDP-N-acetylglucosamine 4-epimerase
3.2.1.46
Galactosylceramidase
5.1.3.12
UDPglucuronate 5´-epimerase
3.2.1.48
Sucrose a-glucosidase
5 1.3.13
dTDP-4-Dehydrorhamnose 3,5-epimerase
3.3.1.1
Adenosylhomocysteinase
5.1.3.14
UDP-N-acetylglucosamine 2-epimerase
3.5.1.1
Asparaginase
5.1.99.1
Methylmalonyl-CoA epimerase
3.5.1.2
Glutaminase
5.2.1.2
Maleylacetoacetate isomerase
3.5.1.6
ß-Ureidopropionase
5.2.1.3
Retinal isomerase
3.5.1.7
Ureidosuccinase
5.2.1.7
Retinol isomerase
3.5.1.8
Formylaspartate deformylase
5.3.1.1
Triose-phosphate isomerase
3.5.1.9
Aryl-formamidase
5.3.1.3
Arabinose isomerase
3.5.1.16
Acetylornithine deacetylase
5.3.1.4
L-Arabinose
3.5.1.18
Succinyl-diaminopimelate desuccinylase
5.3.1.5
Xylose isomerase
3.5.1.19
Nicotinamidase
5.3.1.6
Ribose-5-phosphate isomerase
3.5.1.20
Citrullinase
5.3.1.8
Mannose-6-phosphate isomerase
3.5.1.22
Pantothenase
5.3.1.9
Glucose-6-phosphate isomerase
3.5.1.23
Ceramidase
5.3.1.15
D-Lyxose
3.5.1.32
Hippurate hydrolase
5.3.1.16
3.5.2.2
Dihydropyrimidinase
N-(5´-Phospho-D-ribosylformimino)-5-amino-1(5´´-phosphoribosyl)-4-imidazolecarboxamide isomerase
3.5.2.3
Dihydroorotase
5.3.3.2
Isopentenyl-diphosphate ∆-isomerase
3.5.2.5
Allantoinase
5.3.3.4
Muconolactone ∆-isomerase
3.5.2.7
Imidazolonepropionase
5.3.99.3
Prostaglandin-E synthase
3.5.2.10
Creatininase
5.3.99.5
Thromboxane-A synthase
3.5.3.1
Arginase
5.4.2.1
Phosphoglycerate mutase
3.5.3.4
Allantoicase
5.4.2.2
Phosphoglucomutase
3.5.3.5
Formiminoaspartate deiminase
5.4.2.3
Phosphoacetylglucosamine mutase
3.5.3.6
Arginine deiminase
5.4.2.8
Phosphomannomutase
3.5.4.1
Cytosine deaminase
5.4.3.8
Glutamate-1-semialdehyde 2,1-aminomutase
Guanine deaminase
5.4.99.1
Methylaspartate mutase
AMP deaminase
5.4.99.2
Methylmalonyl-CoA mutase
Methenyltetrahydrofolate cyclohydrolase
5.4.99.5
Chorismate mutase
IMP cyclohydrolase
5.4 99.7
Lanosterol synthase
3.5.4.12
dCMP deaminase
5.5.1.1
Muconate cycloisomerase
3.5.4.19
Phosphoribosyl-AMP cyclohydrolase
5.5.1.4
myo-Inositol-l-phosphate synthase
3.5.4.3
3.5.4.6
3.5.4.9
3.5.4.10
1.5.1.2
(NAD+), L-glutamate-forming
HYDROLASES
dehydratase
4-epimerase
isomerase
ketol-isomerase
3.5.4.22
1-Pyrroline-4-hydroxy-2-carboxylate deaminase
3.6.1.3
Adenosine-triphosphatase
3.6.1.15
Nucleoside-triphosphatase
6.2.1.4
3.6.1.31
Phosphoribosyl-ATP pyrophosphatase
6.2.1.25
Benzoate—CoA ligase
3.7.1.2
Fumarylacetoacetase
6.3.1.1
Aspartate—ammonia ligase
3.7.1.3
Kynureninase
6.3.1.2
Glutamate—ammonia ligase
3.7.1.9
2-Hydroxymuconate-semialdehyde hydrolase
6.3.1.4
Aspartate—ammonia ligase (ADP-forming)
3.9.1.1
Phosphoamidase
6.3.1.5
NAD+ synthetase
6.3.2.1
Pantoate—ß-alanine ligase
6.3.2.2
Glutamate—cysteine ligase
LIGASES
LYASES
Succinate-CoA ligase (GDP-forming)
4.1.1.1
Pyruvate decarboxylase
6.3.2.3
Glutathione synthase
4.1.1.3
Oxaloacetate decarboxylase
6.3.2.5
Phosphopantothenate—cysteine ligase
4.1.1.4
Acetoacetate decarboxylase
6.3.2.6
Phosphoribosylaminoimidazole-succinocarboxamide synthase
4.1.1.9
Malonyl-CoA decarboxylase
6.3.2.7
UDP-N-Acetylmuramoyl-L-alanyl-D-glutamate—lysine ligase
4.1.1.11
Aspartate 1-decarboxylase
6.3.2.8
UDP-N-acetylmuramate—alanine ligase
4.1.1.12
Aspartate 4-decarboxylase
6.3.2.9
UDP-N-acetylmuramoylalanine—D-glutamate ligase
4.1.1.15
Glutamate decarboxylase
6.3.2.10
4.1.1.17
Ornithine decarboxylase
UDP-N-acetylmuramoylalanyl-D-glutamyl-lysine—
D-alanyl-D-alanine ligase
4.1.1.20
Diaminopimelate decarboxylase
6.3.2.13
UDP-N-Acetylmuramoylalanyl-D-glutamate—2,6-diaminopimelate ligase
Phosphoribosylaminoimidazole carboxylase
6.3.3.1
Phosphoribosylglycinamidine cyclo-ligase
Histidine decarboxylase
6.3.4.1
GMP synthase
Orotidine-59-phosphate decarboxylase
6.3.4.2
CTP synthase
Tyrosine decarboxylase
6.3.4.3
Formate—tetrahydrofolate ligase
Aromatic-L-amino-acid decarboxylase
6.3.4.4
Adenylosuccinate synthase
Sulfoalanine decarboxylase
6.3.4.5
Argininosuccinate synthase
Phosphoenolpyruvate carboxylase
6.3.4.7
Ribose-5-phosphate—ammonia ligase
Phosphoenolpyruvate carboxykinase (GTP)
6.3.4.13
Phosphoribosylamine—glycine ligase
4.1.1.33
Diphosphomevalonate decarboxylase
6.3.4.16
Carbamoyl-phosphate synthase (ammonia)
4.1.1.34
Dehydro-L-gulonate decarboxylase
6.3.4.17
Formate—dihydrofolate ligase
4.1.1.36
Phosphopantothenoylcysteine decarboxylase
6.3.5.1
NAD+ synthetase (glutamine-hydrolysing)
4.1.1.37
Uroporphyrinogen decarboxylase
6.3.5.2
GMP synthetase (glutamine-hydrolysing)
4.1.1.38
Phosphoenolpyruvate carboxykinase (pyrophosphate)
6.3.5.3
Phosphoribosylformylglycinamidine synthetase
4.1.1.39
Ribulose-bisphosphate carboxylase
6.3.5.4
Asparagine synthase (glutamine-hydrolysing)
4.1.1.41
Methylmalonyl-CoA decarboxylase
6.3.5.5
Carbamoyl-phosphate synthase (glutamine-hydrolysing)
4.1.1.43
Phenylpyruvate decarboxylase
6.4.1.1
Pyruvate carboxylase
Aminocarboxymuconate-semialdehyde decarboxylase
6.4.1.2
Acetyl-CoA carboxylase
Tartronate-semialdehyde synthase
6.4.1.3
Propionyl-CoA carboxylase
Indole-3-glycerol-phosphate synthase
6.4.1.4
Methylcrotonoyl-CoA carboxylase
4 1.1.21
4.1.1.22
4.1.1.23
4.1.1.25
4.1.1.28
4.1.1.29
4.1.1.31
4.1.1.32
4.1.1.45
4.1.1.47
4.1.1.48
Order 1-800-325-3010
Fax 1-800-325-5052
Technical Service 1-800-848-7791
Customer Service 1-800-325-8070
Quantity Pricing/Standing Orders
Sigma-Aldrich Research 1-800-521-8956 ext. 3702
Development / Manufacturing Inquiries
Sigma-Aldrich Fine Chemicals 1-800-336-9719
Sigma • 3050 Spruce Street, St. Louis, MO 63103 • http://www.sigma.sial.com
The
SIGMA-ALDRICH
Family
Biochemicals and
Reagents for Life
Science Research
Organics and
Inorganics for
Chemical Synthesis
Specialty Chemicals
and Analytical
Reagents for Research
Laboratory Chemicals
and Reagents for
Research and Analysis
Chromatography
Products for Analysis
and Purification
Sigma-Aldrich Research is the research sales and marketing division of Sigma-Aldrich, Inc. Sigma-Aldrich Fine Chemicals is the development and manufacturing division of Sigma-Aldrich, Inc.
©1998 Sigma-Aldrich Co. Printed in USA. Sigma brand products are sold through Sigma-Aldrich, Inc.
Sigma-Aldrich, Inc. warrants that its products conform to the information contained in this and other Sigma-Aldrich publications. Purchaser must determine the suitability of the product(s) for
their particular use. Additional terms and conditions may apply. Please see reverse side of the invoice or packing slip.
SIGMA and
are registered trademarks of Sigma-Aldrich Co. CDM™ is a trademark of Sigma Chemical Company. Riedel-de Haën®: trademark under license from Riedel-de Haën AG.
9038 483-332-432 AFN