GLYCOGEN STORAGE DISEASES

LIVER GLYCOGENOSES

O Glycogen synthase 2.4.1.11

GLYCOPROTEINS
GANGLIOSIDES
MUCINS HYALURONIC ACID
Metabolism
DERMATAN BLOOD GROUP O-ANTIGENS STARCH GLYCOGEN NH
CH2COO-
OH
+
N
CONH2
O

R — O — P — O — P — O— Adenosine(P)

OH OH
O +
N
COO-
O

R — O — P — O — P — O — Adenosine

O-
O

O- NICOTINATE
N
COO-

2.4.2.
11
+
N
COO-
+
N
COO-
COO-
N
COO-
COO-
AMINO ACID METABOLISM
Xanthurenic aciduria
Kynureninase 3.7.1.3
Indoleacetate NH
+ +
SUBSTANCES 2.7.1.23 6.3.5.1 RP 2.4.2.19 RP 2.4.2.19

PEPTIDO CHITIN CHONDROITIN PECTIN MANNAN INULIN CH2OH CH2OH

(Auxin) Indoxyl NADP NAD 6.3.1.5 Desamino-NAD 2.7.7.18 Nicotinic-RP Quinolinate-RP Quinolinate Tryptophanuria
I Glucose-6-phosphatase 3.1.3.9 (von Gierke) CELLULOSE O O COCH2CH(N H3)COO-
+
COCH2CH(N H3)COO-
+
COCH2CH(N H3)COO-
+
COO- COO-
2.4.99.7 GLYCAN O 2.4.1.11
HO
1.2.3.7 Tryptophan 2,3-dioxygenase 1.13.11.11
CH3 CHO NH2 NH2 NH2
O O CH2OH O CH2CHO N OCH NH2 OCH NH2
CHOH O OH OH COO- COO-
III Amylo-1,6-glucosidase (debrancher) 3.2.1.33 (Cori) CHOH COO- CH2OH 2.4.1.16 COO- HO HO OPPG
O OH OH OH H
ACNH 2.4.1.17 3.2.1.33
CH2OH 6.3.2.7-10
O
2.4.1.68
HO CH3 OPPT
OH OH
NH Formyl 3.5.1.9 Kynurenine 1.14.13.9
3-Hydroxy 3.7.1.3
3-Hydroxy 1.13.11.6 2-Amino-3-carboxy 2-Aminomuconate- Hyperserotoninaemia
HO OH OPPU OH NH 4.1.1.45
OPC 6.3.2.13 2.4.1.69 2.4.1.18 Indole- Indole kynurenine kynurenine anthranilate muconate semialdehyde 6-semialdehyde
IV 1,4-Glucan branching 2.4.1.18 (Andersen) HO O OPPU OH GDP-Fucose
HO OH HO OH
2.4.1.29 2.4.1.21
LACTOSE acetaldehyde + Aromatic amino acid decarboxylase 4.1.1.28
O 2.4.1.32 OH OH CH2CH2NH2 HO CH2CH(N H3)COO- HO CH2CH2NH2 HO CH2CH2NHCOCH3 CH3O CH2CH2NHCOCH3
HO
CMP-N-Acetyl 3 CH CH
NHAC UDP- CH3 MANNOSE 2.7.1.38
3.2.1.23
CH2OH 1.13.11.11
O
neuraminate COO- Iduronate O TDP-Rhamnose CH2OH HO NH NH NH NH NH
Hereditary Tyrosinaemia (Type I)
VIII Liver phosphorylase b kinase 2.7.1.38 (Hug) O CH2OH
COO-
HO OPPG
2.7.1.7 CH3 O
4.1.1.43
Tryptamine 5-Hydroxy- 4.1.1.28
5-Hydroxytryptamine
2.3.1.5
N-Acetyl-Serotonin 2.1.1.4 N-Acetyl-5-O-methyl-Serotonin
CHOH
COO-
UDP-N-Ac- O
O
O CH2COCOO- 1.14.16.
4
Fumarylacetoacetase 3.7.1.2
ACNH CHOH Muramate HO
HO
OH
ADP-Glucose
OH OH
4.1.1.28 +
tryptophan (SEROTONIN) COO- COO-
H H
(MELATONIN)
CH2OH CH2O P O CH2CH(N H3)COO-
VI Liver phosphorylase 2.4.1.1 (Hers) 2.7.7.43
OH 3.1.3.29 OH OPPU
GDP-4-Oxofucose O OH
5.1.3.13
OPPT
HO OH
2.4.1.1
OPPU OH NH
4.1.99.1
C—CH(OH)CH(OH)CH2O P HOC—CH(OH)CH(OH)CH2O P NH — CH — C — C — CH— CH2O P
COO- 1.2.1.32
CH2OH
O
OH OPPU CH3 OH GALACTOSE Indole- NH N
CH
N
CH OH OH
NH2
Alcaptonuria
5.1.3.12 O
1.1.1.158 NHCOCH3
OH OH pyruvate 1.4.1 4.2.1.20 H H 4.1.1.48 O 2.4.2.18
HO HO OH HO OH
TDP-4-Oxo- UDP-Glucose CH2OH .19
TRYPTOPHAN Indole-3-glycerol-P 1-(o-Carboxy phenylamino) N-(5-P-ribosyl) anthranilate Anthranilate Homogentisate 1,2-dioxygenase 1.13.11.5
N-Ac-Neuraminate UDP-N-Ac- UDP- O 1.1.1.22 O
COO- COO- COO-
COO-
HO O OPPU
Galactosamine Galacturonate OH HO OPPG Mannose-6-P 6-deoxyglucose 3.2.1.20 HO COO- HO COO- COO- 1-deoxyribulose-5-P
(Sialate) CH2 C 2.7.7.27
2.7.1.6 2.4.1.22
OC CH2 CH2
NHAC COO-
MUSCLE GLYCOGENOSES CH2OH P
O
COO- O GDP-4-Dehydro- 5.4.2.8
CH2OH
CH2OH
O
OH OP P OCH
2
CH2
OH
O — C — COO- OC — COO-
4.1.3.27
Hawkinsuria
5.1.3.6
6-deoxymannose O GDP-Glucose OH
HOCH HCOH
C
OH O
OH
HO
OH
OH P O
OH
OH OP
OH OH
ACNH UDP-N-Ac-Glucosamine 4.2.1.46 O OH 4.2.1.10 1.1.1.25 2.5.1.19 4.6.1.4 4-Hydroxyphenylpyruvate dioxygenase 1.13.11.27
V Muscle phosphorylase 2.4.1.1 (McArdle) HO OH 3.1.3.29 pyruvate 5.1.3.7 HO OH OPPU
CH2OH
HO OH
CH2OH 2.7.7.9 Galactose--P H OH 3-Deoxy-D-arabino- Dehydroquinate Dehydroshikimate Shikimate 2.7.1.71Shikimate-3-P Shikimate-5 Chorismate
OH OPPT CH2OH 4.6.1.3
4.1.3.20
UDP-Glucuronate
O HO OH HO O P O
O
heptulosonic acid 7-P CHO PEP enolpyruvate 3-P
OH OH
N-Ac-Mannosamine-6-P 4.2.1.47
Mannose-1-P TDP-Glucose 2.7.7.34
2.7.7.12 HO COO-
OH -OOC NH2 Hereditary Tyrosinaemia (Type II)
II a-1,4-Glucosidase 3.2.1.20 (Pompe) CH2OH
CH2OH CH2OH HO OH HO OPPG
CH2O P
HO OH OP
OH
COO-
O
O O
O
OH OPPU CH3CH(OH)CH2COCOO- CH2=CH.CH2COCOO- OH 2-Amino Tyrosine aminotransferase 2.6.1.5
PEP 4.1.3.20 5.3.1.8
OH 5.1.3.2
OH 4-Hydroxy-2-oxovalerate
4.2.1.80
2-Oxopent-4-enoate
3.7.1.9
2-Hydroxymuconate Catechol 1.14.12.1 muconate
2.7.1.60 GDP-Mannose 2.7.7.24
VII Muscle phosphofructokinase 2.7.1.11 ACNH
HO OH OH
HO OH OPPU HO OH OP 2.7.7.13 HO OH OH
2.4.1.9 Glucose-1-P
CH2O P CH2OH
UDP-Galactose H2
C
H2
C C
H2 semialdehyde 1.13.11.2
-OOC CH2COCOO-
5.4.99.5
Phenylketonuria
NHAC NHCOCH3 O C COO- C COO- HC COO-
5.1.3.14 COO-
N-Ac-Mannosamine UDP-N-Acetyl N-Ac-glucosamine-1-P N-Ac-glucosamine-6-P NHCOCH3 2.3.1.4 Glucosamine-6-P O O
H2C COO- HC
O
C HC
O
C 1.13.11.1 Phenylalanine 4-monooxygenase 1.14.16.1
COO- 2.7.7.23 COO-
O
glucosamine OH OH OH OP 5.4.2.3
CoA.S.COCH2COCH2CH2COO- C
H2
C
H2 O
C
H O
H H OH 5.3.3.4 5.5.1.1
OH
OH H OH OH OH OH HO OH OH HO OH OH
3-Oxoadipyl-CoA 3-Oxoadipate 3-Oxoadipate Muconolactone Cis,cis-muconate 4.2.1.51
MONO- and DI-SACCHARIDE HOCH2 C C C CO CH2OH 2.4.1.13 2.8.3.6 3.1.1.24
enol-lactone Prephenate Dopamine monooxygenase 1.14.17.1
HOCH2 C C C C COO- 3.2.1.48 NH2 OH O
Fructose
+ +
HO OH OH 3.2.1.26
HO OH HO OH OH CH2COCOO- CH2CH(NH3)COO- CH2CH(NH3)COO- CH2CO COO-
METABOLISM
OH OH H SUCROSE O
H
Gulonate
OH H H
1.1.1.19
Glucuronate
OH 1.13.99.1
Inositol OH 3.1.3.25
OH
Inositol-P
5.5.1.4
H H OH H
GLUCOSE O
O
CH2COO-
CH2COO- 1.3.1.13
Tyrosine-negative Albinism
OH H OH OH -OOC CH2COO- COOH
OH H OH OH OH H OH
GALACTOSE
3.1.1.18 HOCH2 C C C C CH2OH 1.1.1.21
5.4.2.2 ATP 3.7.1.2 1.13.11.5 OH OH OH 1.14.16.1 2.6.1.5
Phenyl Monophenol monooxygenase 1.14.18.1
HOCH2 C C C C CO HOCH2 C C C CO CO HOCH2 C C C C CO 1.1.1.14 OH OH H OH CH2O P Fumaryl 5.2.1.2 Maleyl Homogentisate Hydroxyphenyl 2.6.1.5
TYROSINE PHENYLALANINE
H H H H H H O Sorbitol O
2.7.1.2
2.7.1.1 acetoacetate acetoacetate CH2CH2NH2
1.13.11.27
pyruvate CH2CH2NH2 pyruvate
CHOHCH2NH2 CH2CH2NH2
b-Galactosidase 3.2.1.23 O O H H OH H 2.7.1.
4 O
2.6.1.16 3.1.3.9
ADP
CH2CH(NH2)COO- 4.1.1.53 CHO Hypersarcosinaemia
Gulonolactone 1.1.3.8
2-Oxogulonolactone ASCORBATE 1.10.2.1
P OCH2 C C C C COO- HO OH
CHOHCH2NHCH3 OH 1.14.16.2 4.1.1.25

1.1.1.45 OH H OH OH H OH H 1.10.3.3 CH2O P

O
OH
1.14.17.1 OH 4.1.1.28 OH OH
Sarcosine dehydrogenase 1.5.99.1
OH OH H OH
Galactokinase 2.7.1.6 HOCH2 C C CO C COO- HOCH2 C C CO CO COO- HOCH2 C C CO CO CO 3.1.1.17
OH
1.1.1.49 OH ADRENALINE OH Noradrenaline Dopamine OH Dopa Tyramine Phenyl Benzaldehyde
6-P-Gluconate P-Glucono Glucose-6-P OH
2.1.1.28
2.1.1.6 O
H2
C 1.4.3.4 CH2CHO ethylamine
H OH H H OH H O
lactone HO OH OH
CH.COO -
CH(OH)CH2NH2 Non-ketotic hyperglycinaemia
Dehydroascorbate NADP+
CH2CHO
3-Dehydrogulonate 2, 3-Dioxogulonate 2.7.1.3
.3.
4 1.14.18.1
1.14.17.1 COO-
Galactose-1-phosphate uridylyltransferase 2.7.7.12 H H OH OH CH(OH)COO- 1.4 CH(OH)COO- O
NH3
+
1.4.3.4 1.2.1.7 Glycogen Cleavage System
4
OH H OH H H OH H H 1.1.1.44 1.4.3. CHOHCH2NH2
HOCH2 C C C CO CH2O P
Dopaquinone OH OH
Glycine dehydrogenase (decarboxylating) 1.4.4.2
UDP-Galactose 4-epimerase 5.1.3.2 HOCH2 C C C CHO HOCH2 C C C CHO HOCH2 C C CO CH2OH OH OH H Fructose-1-P NADPH 5.3.1.9
OH
OH

3, 4-Dihydroxy- OH
OCH3
OCH3
Hydroxyphenyl-
ethanolamine
Hydroxyphenyl-
acetaldehyde
Phenyl
acetaldehyde
Benzoate
H OH H
4.1.1.34
OH OH H
5.3.1.3
OH OH
D-mandelate 4-Hydroxy-3-methoxy- OH
1.14.18.1
CH(OH)CHO CH2COO-
1.14.13.12 Aminomethyltransferase 2.1.2.10
H H HO +
CH2CH(NH3)COO- CH2COO-
L-XYLOSE D-ARABINOSE D-RIBULOSE 2.2.1.1 D-mandelate Normetadrenaline COSCoA
6.2.1.25
Sorbitol dehydrogenase deficiency 1.1.1.14 P OCH2 C C C CO CH2OH (VMA) H
1.4.3.4
1.2.1.3 1.2.1.39

HOCH2
OH H
C C
H
C CH2OH HOCH2
OH H
C C CO CH2OH OCH2
OH H
C C CO CH2OH 2.7.1.47 4.2.1.12 OH OH H
CH(OH)CH2OH N O
I I
OH
PURINE & PYRIMIDINE
P OH
Primary sucrase-isomaltase deficiency CH(OH)CH2OH O Hydroxyphenyl Hydroxyphenyl Phenyl acetate Benzoyl-CoA METABOLISM
H
L-Arabitol
OH OH H
L-Xylulose
OH
2.7.1.53
H
L-Xylulose-5-P
OH 5.1.3.1
Fructose-6-P OH
OCH3
O
O
glycolaldehyde
CH(OH)COO-
acetate - COO
CO.NHCH2COO-
Sucrase 3.2.1.48 OH
4-Hydroxy-3-methoxy
OH OH H H OH H H OH H H H
ATP OH
3, 4-Dihydroxy phenylglycol
I I 1.2.1.3 3.5.1.32 Xanthinuria
HOCH2 C C C CHO HOCH2 C C C CH2OH HOCH2 C C C CHO P OCH2 C C CO CH2OH H H 2.1.1.6 O N OH OH
3.1.3.11 2.7.1.11 phenylglycol (MOPEG) H 1.14.13.12
Xanthine oxidase 1.1.3.22
Pentosuria H H OH OH H OH OH H OH OH OH 2 P OCH2 C C CHO MELANIN THYROXINE OH
Coenzyme-Q Hydroxybenzoate Hippurate
1.1.1.10 2.7.1.47
ADP N
Hydroxy-
L-Xylulose reductase 1.1.1.10 L-Arabinose Xylitol D-Xylose D-Ribulose-5-P OH OH
Erythrose-4-P H2C
NH
H2C
NH
HC
-OOC N mandelate -OOC.CH.CH COO-
2
N
O
C N
O
NH2 C C
N
OH H H 5.3.1.4 OH OH OH OH H H HO CH2O P
OC
CHO
HN C
CHO
H2N C
CH C
CH HNCOC
CH
NH2 C
CH
CH Primary Gout
O NHCOCH2NH2 C H2N C C HCO
HOCH2 C C C CHO HOCH2 C C CO CH2OH OCH2 C C CO CH2OH 5.1.3.1 NH NH N N N RP
FRUCTOSE P
P OCH2 C C C CO CH2O P 2.1.2.2 RP 6.3.5.3 RP 6.3.3.1 RP 4.1.1.21 H2N RP 6.3.2.6
N RP
4.3.2.2 NH2
N RP 2.1.2.3
N
H I AMP deaminase 3.5.4.6
H OH OH H H H H H OH OH OH H Glycinamide- Formyl Formyl 5-Aminoimidazole-RP 5-Amino-4-imidazole 5-Amino-4-imidazole 5-Aminoimidazole Formylamido-
L-LYXOSE L-RIBULOSE L-Ribulose-5-P 5.1.3.4 5.3.1.6
4.1.2.- ribosyl-P glycinamide-RP glycinamidine-RP carboxylate-RP (N-succinylcarboxamide)-RP carboxamide-RP imidazole- II Hypoxanthine phosphoribosyltransferase 2.4.2.8
Essential fructosuria H OH OH
2.7.1.16
H OH
P OCH2 C
OH H
C CO CH2OH
2.2.1.2
Fructose- 6.3.4.13
OH OH
H2NCONH2
H
NH2 COO- NH2
H2N OC
NH O
C
C
N O
C
N O
C
N O
N
carboxamide-RP III Ribosephosphate pyrophosphokinase 2.7.6.1
Ketohexokinase 2.7.1.3 HOCH2 C C C CHO
1.1.1.9 HOCH2 C C CO CH2OH
2.7.1.17
D-Xylulose-5-P 1:6-bis-P 3.5.3.4
Urea OC C
N H
CO
NH
OC C
N H NH
CO
OC
HN

N
C
NH
CO HN
OC
C
C
CH HN
HC
C
C
CH
2.4.2.1 Asparate
HN
C
C
CH 3.5.4.10
OH H H OH H H 3.5.2.5 H N NH 1.1.3.22 NH HC
1.7.3.3 H 1.1.1.204 N
D-LYXOSE
5.3.1.15
D-Xylulose CH2(NH2)COOH
Allantoate Allantoin URATE
H
Hypoxanthine
2.4.2.8 N N RP
Adenylosuccinate lyase 4.3.2.2
Xanthine INOSINE-P
Fructokinase deficiency 2.7.1.4 H OH OH H H H H H H GLYCINE NH2 NH2
1.1.3.22
NH2 N
-OOC.CH.CH COO-
2
H H H H H H OH CH2O P
1.4.1.10
N
N N C Fumarate NH 6.3
.4.4 (IMP)
HOCH2 C C C CH2OH HOCH2 C C C CH2OH HOCH2 C C C CHO
P OCH2 C C C CHO
O NH2
2.6.1.4 1.5..3.1
O
N N C
CH C N 3-Aminoisobutyric aciduria
P OCH2 C C C C CO CH2O P 1.5.99.1 N O O O
Fructose-1,6-bis-phosphatase deficiency 3.1.3.11 OH H H OH OH OH OH OH OH 2.1.1.20 N N N
3.6.1.3 HC C N C
CH
3-Aminoisobutyrate aminotransferase 2.6.1.22
D-Arabitol Ribitol D-RIBOSE
2.7.1.15 OH OH OH OH OH OH H
4.1.2.13
-OOCCH NHCH
-O P O CH2 O -O P ~O P ~O P O CH2 O 2.7.4.6 2.7.4.3
N N RP
HC C
O
C N 1.1.1.205
D-Ribose-5-P Sedoheptulose-P OH OH
2

Sarcosine
3

4.6.1.1 O- O- O- ADP ADENOSINE-P 4.3.2.2 Adenylosuccinate

N NH RP HN C
CH
Fructose intolerance PHOTOSYNTHESIS 1.5.99.2 O
2.7.4.4 (AMP) OC C
Thymine-uraciluria
4.1.2.9 P-Ribosyl 1.4.4.2 OH OH OH
3.5.4.6 N N RP

Fructose-1.6-bis-phosphate aldolase (isoenzyme B) 4.1.2.13

H H
amine
-OOCCH N(CH )
2 3 2
2.1.2.10
Cyclic AMP
ATP 2.4.2.1 H
XANTHOSINE-P
ATP 2.7.1.19
P OCH2 C C CH2 CO COO- P OCH2CHOHCHO
Dimethylglycine 1.17.4.1
2.7.7.7 2.7.7.7
2.4.2.15, 3.5.4.3
(XMP)
Dihydropyrimidine dehydrogenase 1.3.1.2
Photo- ADP OH OH
2.2.1.1 3-P-Glyceraldehyde 2.1.1.5 d-ADP
2.7.4.6
d-ATP DNA d-GTP d-GDP O
C N
PENTOSE PHOSPHATE PATHWAY System Chlorophyll 2-Oxo-3-deoxy- 2.7.6.1 +
-OOCCH N (CH )
2.7.7.7 2.7.7.7
HN C
CH
6.3.4.1
Orotic aciduria
II 6-P Gluconate 2.4.2.14 2 3 3 TTP 1.17.4.1 H2NC C
6.3.5.2
HOCH2COCH2O P
Chlorophyll .1 Pi 6.3.4.7 Betaine FOLIC
N N RP
Orotate phosphoribosyltransferase 2.4.2.10
5.3.1
REDOX POTENTIAL
–

Phosphogluconate dehydrogenase (decarboxylating) 1.1.1.44

Pheophytin
Ferredoxin 4.1.2.13
Dihydroxy Pi NAD+
1.2.1.8
ACID
2.7.4.6 2.7.7.6
RNA
2.7.7.6
GTP GDP
2.7.4.4
GUANOSINE-P
Orotidine-5´-phosphate decarboxylase 4.1.1.23
2.1.2.1 C1 2.7.4.6

Glucose-6-phosphate dehydrogenase 1.1.1.49 2e-

4.1.2.14
acetone-P +
OHCCH2N(CH3)3
POOL
TDP
O
(GMP)
PQ
2e- NADP+ Betaine O
C
O
C
O C
d-CTP
b6 1.2.1.12
1.1.99.1 aldehyde HN CH.CH3 HN C.CH3 2.7.4.9
HN
C
C.CH3
HN CH
2.7.4 FOLIC ACID
H2O Cytochrome 1.2.1.13 HOCH2CH(NH2)COO-
H2NCONHCH2CHCOO-
CH3
OC CH2 OC CH
OC CH
2.7.7.6 2.7.7.6 OC
N
CH .6

GLUCONEOGENESIS complex N N DP
2e- NADH 6.3.4.3 SERINE H 1.3.1.2 H 2.4.2.4
N
DP 3.5.4.12 2.7.4.14
Plasto f
PC NADPH 6.3.4.17
CH3

H2NCH2CHCOO-
3.5.1
.6
ß-Ureido
isobutyrate
3.5.2.2
Dihydro
thymine
Thymine
O O
THYMIDINE-PNH 2.1.1.45
d-UMP d-CMP d-CDP 5,10-Methylenetetrahydrofolate reductase 1.7.99.5
Glucose-6-phosphatase 3.1.3.9 quinone 1.10.99.1 H 2.7.1.28 2.6.1.51 C C C
2
+

1.4.1.7
Mn Plasto H H ß-NH2-Isobutyrate HN CH2 HN CH N CH
cyanin P700 P OCH2 C C CO CH2O P
HOCH2 C CHO P OCH2CHOHCOO P
HOCH2COCOO- 4.1.1.11 OC CH2 OC CH OC CH 1.17.4.1 Dihydrofolate reductase 1.5.1.3
Fructose-1,6-bis-phosphatase 3.1.3.11 P680 Photo- OH OH Glyceraldehyde
OH
1:3-bis-P-Glycerate Hydroxy-
pyruvate 3.1.3.3
H2NCH2CH2COO-
ß-Alanine
3.5.1.6
H2NCONHCH2CH2COO-
Carbamoyl
3.5.2.2
Dihydrouracil
N
H 1.3.1.2
N

Uracil
H 3.5.4.1
Cytosine
N
H 2.4.2.1
CDP
system
I
Ribulose-1:5-di-P -OCO O
C
ß-alanine O
C
O
C
O
C
O
C NH2
Glutamate formiminotransferase 2.1.2.5
Phosphoenolpyruvate carboxykinase (GTP) 4.1.1.32 2H+ CH2O P ADP HN CH C
1/2 O2 Light-driven electron flow from H2O to NADP+
CO2 Fixation
P-Ribosyl-PP
O 1.1.1.29
1.1.1.81
P OCH2CH(NH2)COO-

Phospho-
H2N
OC
N
CH2
CH.COO-
HN
OC
N
CH2
C.COO-
HN
OC
N
CH
C.COO-
HN
OC
N
CH
C.COO-
2.4.2.9 NH
OC
N
CH
CH OC
N
CH
3.6.1.15
2.7.4.6
N
OC
CH
CH AMINO ACIDS
2.7.2.3 2.6.1.22 RP RP RPPP
4.1.1.39 OPP serine H 3.5.2.3
H
1.3.1.14
H 2.4.2.10 4.1.1.23 2.7.4.4 2.7.4.6 6.3.4.2 N
Pyruvate carboxylase 6.4.1.1 HO HO Carbamyl Dihydro Orotate Orotidine-P Uridine-P UDP UTP CTP RPPP

COO- COO-
OH OH
ATP 2.6.1.52 aspartateH H H H
orotate NH 2 H H H H NH2 (UMP) H H CONH2 N H H Cystathioninuria
COO- 2.6.1.18 N N
C OC
NON-SPHEROCYTIC P OCH2CHOHCOO- P OCH2COCOO-
P OCH2 C C C C N
+
C
CH
P OCH2 C C C C NH C
CH
P OCH2C C CO CH2 NH C
CH
P OCH2 C C C CH Cystathionine lyase 4.4.1.1
1.13.11.34
Linoleate 1.14.99.25 g-Linolenate HC C HC C HC C OH OH HN N
HAEMOLYTIC ANAEMIAS DUE TO
COO-
Arachidonate
O
Leucotriene B4 O 3-P-Glycerate 1.1.1.95
P-hydroxy-
pyruvate
3.5.1.7
2.1.3.2
2.4.2.17
OH OH
O
N N
RPPP
OH OH
O
N N
RPP
OH OH

P-Ribulosylformimino
N N
RP
C
H
ERYTHROCYTE ENZYMOPATHIES 1.3.1.35
5.3.99.3
1.14.99.1 COO- COO- P-Ribosyl-ATP P-Ribosyl-AMP P-Ribosylformimino Imidazole glycerol-P Histidinaemia
CO.S-ACP 2.7.1.31 HCOO- 3.6.1.31 3.5.4.19
5-Aminoimidazole- 5.3.1.16 5-Aminoimidazole-
COSCoA
HOCH2CH(OH)COO- Formate -OOC CHCH2COO- carboxamide-RP
4.2.1.19 Histidine ammonia-lyase 4.3.1.3
HO
O -OOCCHCH COO- carboxamide-RP
Hexokinase 2.7.1.1 Oleoyl-CoA Palmitoleoyl-ACP HO OH
5.3.99.5
OH P OCH2CH(O P )COO- Glycerate 2
OC CHCH2COO- HC C — CH2COO- HC C — CH2CHO HC C CH2COCH2O P
Prostaglandin PGE2 Thromboxane B2 5.4.2.1 NHCHO
HN NH
Imidazole acetol-P
1.14.99.5
COSCoA CH3(CH2)14CH=CHCOSCoA CH3(CH2)14CH(OH)CH2COS.CoA CH3(CH2)14COCH2COS.CoA
2, 3-Diphospho- C
H
N NH N NH N NH N
C
NH
Homocysteinaemia
glycerate CH3COO P 3.5.1.8 N-Formyl C C C
H 2.6.1.9
Glucose-6-phosphate isomerase 5.3.1.9 Stearoyl-CoA Dehydrostearoyl-CoA OH-Stearoyl-CoA Oxostearoyl-CoA aspartate Formimino H Imidazolone H
Imidazole Imidazole
H
Cystathionine synthase 4.2.1.22
ACETYL-P aspartate acetate 1.14.13.5 acetaldehyde HC C CH2CH(NH2)CH2O P
Fatty Acyl Synthase - 2.3.1.85 - includes
Microsomal
3.5.3.5 acetate
Histidinol-P
6-Phosphofructokinase 2.7.1.11 CH3(CH2)14COS-ACP
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)14COSCoA
HOCH2CHO P COO-
2.7.2.1
SO42- -OOC CHCH2CH2COO- OC CHCH2CH2COO-
HC C CH2CH2NH2
N
C
H
NH
3.1.3.15
5-Methyltetrahydrofolate-homocysteine
Palmitoyl-ACP Palmitoyl-CoA Chain elongation Mitochondrial
2-P-Glycerate 1.8.2.1
1.8.3.1
2.7.7.4
2.7.1.25
H2N
C
O
NH HN
C
O
NH
N
C
NH
HC C CH2CH(NH2)CH2OH
methyltransferase 2.1.1.13
CH3(CH2)n+2CO.S-ACP CH3(CH2)nCH=CHCO.S-ACP CH3(CH2)nCH(OH)CH2CO.S-ACP CH3(CH2)nCOCH2CO.S-ACP
Fructose-1,6-bis-phosphate aldolase 4.1.2.13 1.3.1.9 4.2.1.60 1.1.1.100 CH3COO- P-Adenyl-SO4 Carbamoyl glutamate Hydantoin-5- H
HISTAMINE N NH Histidinol
ACYL-ACP 1.3.1.10 2, 3-Enoyl-ACP 4.2.1.61
3-OH-Acyl-ACP 3-Oxoacyl-ACP propionate C
1.1.1.23 Glutamylcysteine synthase deficiency
2.3.1.41 ACETATE 1.8.99.4 4.1.1.22 H

Triose phosphate isomerase 5.3.1.1 Glutamate-cysteine ligase 6.3.2.2

HSO3-
4.2.1.11 -OOC CHCH2CH2COO- OC CHCH2CH2COO- CH C CH CHCOO- +
CH3(CH2)6CH2CH2COS.ACP CH3(CH2)5CH=CHCH2COS.ACP HC C CH2CH(N H3)COO-
CH3(CH2)6CH(OH)CH2COS.ACP CH3(CH2)6COCH2COS.ACP HN NH N NH N NH
Decanoyl-ACP 3, 4-Decenoyl-ACP 4.2.1.60
3-Hydroxy- 1.1.1.100
3-Oxo-decanoyl-ACP
1.8.99.1
CH CH CH N N
HC C
+

Glyceraldehyde-3-phosphate dehydrogenase 1.2.1.12

2.1.2.5 C
CH2CH(N H3)CHO
Hypermethioninaemia
HS-
CH3(CH2)6CH=CHCOS.ACP
1.3.1.9 decanoyl-ACP 2.3.1.41 2.3.1.30 Formimino Imidazolone Urocanate H Histidinal
2, 3-Decenoyl-ACP 4.2.1.60
CH2 C(O P )COO- CH3CH2OH glutamate 3.5.2.7
propionate 4.2.1.49 4.3.1.3 HISTIDINE 1.1.1.23
N
C
NH
Methionine adenosyltransferase 2.5.1.6
4.2.99.8 H
Adenylate kinase 2.7.4.3 CH3(CH2)2CH2CH2CO.S-ACP
Hexanoyl-ACP
1.3.1.9
CH3(CH2)2CH=CHCO.S-ACP
4.2.1.59
CH3(CH2)2CH(OH)CH2CO.S-ACP
3-Hydroxy- 1.1.1.100
CH3(CH2)2COCH2COS.ACP P-enolpyruvate ETHANOL S — CH2CH(N H3)COO-
+

CH2CH(N H3)COO-
+

2, 3-Hexenoyl-ACP 3-Oxo-hexanoyl-ACP S — CH2CH(N H3)COO-

+
HSCH2CH(N H3)COO-
+
4.4.1.1 + 4.2.1.22 + CH3SCH2CH2CH(N H3)COO-
+
Propionic acidaemia
hexanoyl-ACP 2.3.1.41 1.6.4.1 SCH2CH2CH(N H3)COO- HSCH2CH2CH(N H3)COO-
2.1.1.13
Phosphoglycerate kinase 2.7.2.3 ADP
3
CYSTINE CYSTEINE Cystathionine
4.4.1.8
Homocysteine 2.1.1.14 METHIONINE Propionyl-CoA carboxylase 6.4.1.3
.1.1
CH3CH2CH2CO.S-ACP CH3CH=CHCO.S-ACP CH3CH(OH)CH2COS-ACP CH3COCH2CO.S-ACP 2.7.1.40 4.2
1.3.1.9 4.2.1.58 1.1.1.100 1.13.11.20
Phosphoglycerate mutase 5.4.2.1 Butanoyl-ACP Crotonoyl-ACP 3-OH-Butanoyl-ACP Acetoacetyl-ACP HOOCCH2CO.S-ACP HSCH2CH2NH2 Adenosyl 3.3.1.1 Adenosyl
2.5.1.6

CH2OH CH2OH Malonyl-ACP ATP 4.4.1.1 Cysteamine + + + CH3 + + Erythrocyte glutathione synthase deficiency
CH3(CH2)n+2CO.S-CoA R.CH2COO- 1.1.1.8 HO2SCH2CH(N H3)COO- HO3SCH2CH(N H3)COO- SCH2CH2CH(N H3)COO- SCH2CH2CH(N H3)COO-
1.1.1.1
6.2.1.3 2.3.1.41
Cysteine sulphinate Cysteate S-Adenosyl S-Adenosyl Glutathione synthase 6.3.2.3
Pyruvate kinase 2.7.1.40 ACYL-CoA 3.1.2.20 FATTY ACID HOCH HOCH
2.3.1.39 4.1.1.29 homocysteine 2.1.1.20 2.1.1.10
methionine
(Cytosol)
CH2OH 2.7.1.30 CH2O P
KETONE BODIES 3.7.1.2 CH3CHO 4.1.1.29 (SAM) Hydroxyisobutyryl-CoA hydrolase 3.1.2.4
Glycerol 3-P-Glycerol 4.1.1.1 4.1.2.5
Glutamate 6.3 +
-OOCCH(N H )CH CH CONHCHCOO-
Hyperoxaluria 2.3.1.7 CH3COCH3 CH3CH(OH)CH2COO- CH3COCOO- Acetaldehyde .2.2 3 2 2 +
-OOCCH(N H )CH CH CONHCHCONHCH COO-
4.2.99.9 CH2SH
3 2 2 2
HO2SCH2COCOO- Methylmalonic acidaemia
Carnitine O.CO.R
CH2O.CO.R CH2O.CO.R CH2O.CO.R
2.3.1.15
2.3.1.51
ACETONE 3-OH-Butyrate
4.1.1.4
1.1.1.30
HOOCCH2CO.SCoA
Malonyl-Co-A PYRUVATE 1.4.1.1
CH3CH(N H3)COO-
+ 3-Sulphinyl
HO2SCH2CH2NH2
Hypotaurine
1.8.1.3
HO3SCH2CH2NH2
Taurine
g-Glutamylcysteine
6.3.2.3
Glutathione CH2SH

Type II Glycerate dehydrogenase 1.1.1.29

+
(CH3)3NCH2CH.CH2COO-
R.CO.OCH
FAT R.CO.OCH R.CO.OCH
CH3COCH2COO-
1.2.4.1 2.6.1.2
pyruvate Glycine Methylmalonyl-CoA decarboxylase 4.1.1.41
O-Acyl-carnitine 3.1.1.3 CH2O.CO.R" CH2OH CH2O P
Acetoacetate
2.3.1.12
1.8.1.4
ALANINE HOOCCH2CH2CO.O

Triacylglycerol Diacyl Phosphatidate CH3CO.S-ACP

4.1.1.9 4.1.1.12
CH2CH2CH(N H3)COO-
+ +
HOCH2CH2CH(N H3)COO- Bile Acids Methylmalonyl-CoA mutase 5.4.99.2
3.1.1.28
Type I Glycolic aciduria 2.3.1.20 glycerol 3.1.3.4
Acetyl-ACP OHCCH2COO- Succinylhomoserine Homoserine
2.7.1.107
3.1.2.11
2.8.3.5 4.1.1.12
Malonic 2.3.1.46 Methylmalonyl-CoA epimerase 5.1.99.1
Alanine: glyoxylate aminotransferase 2.6.1.44 CH3(CH2)nCH2CH2COSCoA
CH3(CH2)nCH=CHCOSCoA CH3(CH2)nCH(OH)CH2COSCoA CH3(CH2)nCOCH2COSCoA ATP
1.1.1.27
semi- [ O.P.P.-Adenosine-3´- P CH2C(CH3)2CH(OH)CONHCH2CH2CONHCH2CH2SH
2.3.1.38 CH3CH(OH)COO- + +
ACYL-CoA 2, 3-Enoyl-CoA 3-OH-Acyl-CoA
1.1.1.35
3-Oxoacyl-CoA GTP 1.1.1.39
LACTATE
aldehyde CH3COCHO CH3COCH2NH2
1.1.1.103
CH3CHOHCH(N H3)COO- P OCH2CH2CH(N H3)COO-
Coenzyme A Maple Syrup Urine
(Mitochondria) 1.3.99.3 4.2.1.17 2.3.1.16 1.2.1.23
Methylglyoxal Aminoacetone THREONINE O-Phospho- 1.1.1.3
4.2.99.2
homoserine Brached -chain oxo-acid dehydrogenase 1.2.1.25
LIPID METABOLISM 4.1.3.18
4.1
.3.1
8
COO-
CH3
C(OH)CH(OH)COO-
2.7.1.24
CH3(CH2)2CH2CH2COSCoA CH3(CH2)2CH=CHCOSCoA CH3(CH2)2CH(OH)CH2COSCoA CH3(CH2)2COCH2COSCoA CH3 CH3
[ O.P.P.-Adenosine
4.2.1.17 1.1.1.35 1.2.1.25 +
1.3.99.3 CH3COC(OH)CH2CH3
CH3COSCoA CH3CH2 CHCOCOO- CH(N H3)COO-
Hexanoyl-CoA 2, 3-Hexenoyl-CoA 3-OH-Hexanoyl-CoA 3-Oxohexanoyl-CoA 2.3.1.16 1.2.1.18
2-Aceto-2- CH C(CH ) CH(OH)CONHCH CH CONHCH CH SH
2 3 2 2 2 2 2 Hypervalinaemia
Acylcarnitine hydrolase 3.1.1.28 ACETYL-CoA 2:3-di- CH3CH2 CH3CH2
Dephospho-Coenzyme A
2.3.1.9 Hydroxy- hydroxy-3 4.2.1.9 2-Oxo-3-methyl ISOLEUCINE Valine aminotransferase 2.6.1.32
CH3CH2CH2COSCoA CH3CH=CHCOSCoA CH3CH(OH)CH2COSCoA CH3COCH2COSCoA 4.2.1.52 CH3CH2COCOO-
1.3.99.2 4.2.1.55 1.1.1.157
butyrate 1.1.1.86 methylvalerate valerate 2.6.1.42
Butanoyl-CoA Crotonoyl-CoA 3-OH-Butanoyl-CoA Acetoacetyl-CoA Oxobutyrate 2.7.7.3
Acid lipase 3.1.1.3 2.3.1.16 CH3 CH3 CH3 CH3
Odd C Fatty acids CH3COCHCOSCoA CH3CHOHCHCOSCoA CH3CH = CCOSCoA CH3CH2CHCOSCoA P OCH2C(CH3)2CH(OH)CONHCH2CH2CONHCH2CH2SH 3-Methylcrotonylglycinuria
CH3(CH2)3COSCoA CH3CH2CH=CHCOSCoA CH3CH2CH(OH)CH2COSCoA 1.1.1.35 CH3CH2COCH2COSCoA 2-Methyl 2-Methyl-3- Tiglyl-CoA 2 Methylbutyryl 4-P-Pantetheine
Triacylglycerol lipase 3.1.1.3 Pentanoyl-CoA Pentenoyl-CoA 3-OH-pentanoyl-CoA 3-Oxopentanoyl-CoA 4.1.3.5 6.4.1.1
CH3
acetoacetyl-CoA
1.1.1.35
Hydroxy- 4.2.1.17 1.3.99.3
CoA
Methylcrotonyl-CoA carboxylase 6.4.1.4
OHCCHCOO- 4.1.1.36 COO-
CH2OR CH2OR -OOCCHO COO- CH butyryl-CoA 3 CH3 CH3 +
OH OH O COO- CH2OR 2.7.7.41 2.7.8.5 Methylmalonyl C(OH)CH(OH)COO- CHCOCOO- CHCH(N H3)COO- P OCH2C(CH3)2CH(OH)CONHCH2CH2CONHCHCH2SH
Pancreatic lipase 3.1.1.3 R'OCH O
ROCH 3.1.3.27 Glyoxylate 1.4.1.10
semialdehyde CH3COC(OH)CH3 CH3 2.6.1.32 CH3 Isovaleric acidaemia
+ R'OCH CH3 4-P-Pantothenylcysteine
CH2OPO
CH2OPOCH2CHNH3
O- Serine CH2OPPC
1.1.3.15
1.1.1.79 CH2COO-
2.6.1.4
2.6.1.44
4.1.1.32
2-Acetolactate 2-3-dihydroxy 2-Oxo- VALINE 1.2.1.25
Isovaleryl-CoA dehydrogenase 1.3.99.10
Long-chain acyl-CoA dehydrogenase 1.3.99.3 4.1.3.4 1.1.1.86 isovalerate 4.2.1.9 isovalerate 2.6.1.32 Cysteine 6.3.2.5
O- HO OH PHOSPHATIDYL CDP-diacyl 1.2.3.5
HOCH2COO-
CH3C(OH)CH2COSCoA -OOCCOCH COO- 1.1 CH3 CH3 CH3 CH3
2.7.8.8 2
OCH2C (CH3)2CH(OH)CONHCH2CH2COO-
SERINE Inositol glycerol ß-OH-ß-Methyl- .1.3 P

Phosphatidyl
OH
Glycolate glutaryl-CoA
OXALOACETATE 1
HOCH2CHCOO-
3-Hydroxy-
HOCH2CHCOSCoA CH2 = CCOSCoA CH3CHCO.SCoA
4-P-Pantothenate 4-Hydroxybutyric acidaemia
Medium-chain acyl-CoA dehydrogenase 1.3.99.3 R'OH2C 2.7.8.11 1.2.1.21 3-Hydroxy- Methylacrylyl- Isobutyryl-CoA
inositol
CH2OR'
HCOR
CH2OR' HOOC.COOH 1.1.1.34 isobutyrate 3.1.2.4 isobutyryl 4.2.1.17
CoA 1.3.99.3 2.7.1.33 Succinic semialdehyde dehydrogenase 1.2.1.16
4.1.1.65 ROCH O O
ROCH O
Oxalate HOCH2CHO 4.1.3.8
1.1.1.37
CoA HOCH2C (CH3)2CH(OH)CONHCH2CH2COO-
Short-chain acyl-CoA dehydrogenase 1.3.99.2 CH2OR
CH2OPOCH2CHOHCH2OPOCH2
Glycol CH2COO- 4.1.3.7 -OOCCH(OH)CH COO- COOH COOH 1.2.1.25
PANTOTHENATE
CH2OPOCH2CHOHCH2OH CH2COO- 2
R'OCH O O- O- O-
aldehyde C(OH)COO- MALATE (CH3)2CHC(OH)CH2COO- (CH3)2CHCHCH(OH)COO- (CH3)2CHCH2COCOO- (CH3)2CHCH2CH(N H3)COO-
+
3.5.1.22
Aminoadipic aciduria
CH2OP OCH2CH2NH3
+ Cardiolipin Phosphatidyl-glycerol 1.4.3.8
CH3C(OH)CH2CHO
CH3CH2COSCoA ß-Alanine 6.3.2.1
3-Hydroxyacyl-CoA dehydrogenase 1.1.1.35 O- CPP OCH2CH2NH3
+
P OCH2CH2NH3
+ +
HOCH2CH2NH3
Mevaldate CH2COO-
4.1.3.2 Propanoyl-CoA
2-Isopropyl- 3-Isopropyl- Oxoleucine LEUCINE HOCH2C (CH3)2CH(OH)COO-
2-Aminoadipate aminotransferase 2.6.1.39
HOOCCHO malate 4.2.1.33 malate 1.1.1.85
Phosphatidyl CDP ethanolamine Ethanolamine-P
2.7.1.82
Ethanolamine 1.1.1.32
CITRATE Glyoxylate
4.2.1.2
-OOCCH=CHCOO-
1.4.3.1
2.1.3.1 6.4.1.3 CH3 CH3
2.6.1.6
Pantoate
Acetyl-CoA C-acyltransferase 2.3.1.16 ethanolamine 2.7.8.1 2.7.7.14 4.1.1.41 -OOCCH C = CHCOSCoA CH3C = CHCOSCoA (CH3)2CHCH2COSCoA CH3 Hyperpipecolic acidaemia
CEPHALIN CH2OR CH2OH CH3COCH2CH2N(CH3)3
+ 4.2.1.18
FUMARATE 5.1.99.1 2

3-Methylglutaconyl-CoA 3-Methylcrotonyl-CoA Isovaleryl-CoA

HCHO 1.1.1.169
HOCH2 — C — CO — COO-
Pipecolate dehydrogenase 1.5.99.3
HOCH O HOCH O Acetylcholine CH3
6.4.1.4
1.3.99.10
Hydroxymethylglutaryl-CoA synthase 4.1.3.5 CH2OPOCH2CH2N(CH3)3
+
CH2OPOCH2CH2N(CH3)3
+ 1.1.99.1
CH2COO-
4.2.1.3
4.3.1.1 -OOC.CH.COSCoA
CH3 Oxopantoate
4.1.2.12
O- O- 3.1.4.2 NHCOCH2CH2COO-
2.1.1.17 Lysolecithin Glycerophospho- 2.3.1.6 CH3C(OH)CH2CH2OH 1.3.5.1 Methylmalonyl-CoA H
C
H2
C
NHCOCH2CH2COO- +
N H3
Glutaric aciduria (Type I)
2.1.1.71 CH2OR 3.1.1.5 -OOCCH.CH CH CH CHCOO-
choline CHOHCOO- D L (B12) HC CH2 H2C CH2 -OOCCOCH CH CH CH.COO-
Hydroxymethylglutaryl-CoA lyase 4.1.3.4 CH2OCH CHR
Mevalonate -OOC.CH CH COO-
2 2 2
2 2 2
-OOCCHCH CH CH CHCOO- Glutaryl-CoA dehydrogenase 1.3.99.7
ROCH O
R'OCH O
2.7.1.36 CHCOO- 2 2 + + -OOCC CH.COO- -OOCC CH.COO- + 2 2 2
+ -OOCCH CH(N H )COO-
+ P OOCCH2CH(N H3)COO- OHCCH2CH(N H3)COO- N N N H3 +

SPHINGOLIPIDOSES
CH2OPOCH2CH2N(CH3)3
+ CH2O P
O-
OCH2CH2N(CH3)3
CPP OCH2CH2N(CH3)3
+
P OCH CH N(CH )
2 2 3 3
+

2.7.1.32
HOCH2CH2N(CH3)3
+ 2.7.4.2
CH2COO- SUCCINATE ASPARTATE
2 3

2.7.2.4
Aspartyl-P 1.2.1.11
Aspartyl 2, 3-Dihydro- 1.3.1.26 Piperideine-2, 6- N-Succinyl-2- N-Succinyl-2,6- Diamino-
2.6.1.17 N H3

O- 2.7.7.15
Choline-P 4.1.3.1
semialdehyde
4.2.1.52
dipicolinate dicarboxylate amino-6-oxopimelate diaminopimelate 3.5.1.18 pimelate Saccharopinuria
Choline plasmalogen LECITHIN 2.7.8.2
CDP-choline
3.1.4.3
CHOLINE
CH2COO-
ISOCITRATE GTP
+
NH3
O O 6.2.1.4
3.1.2.3 CH2CH2COO- CH2CH2COO- COO-
Saccharopine dehydrogenase 1.5.1.9
Acylsphingosine deacylase (Ceramidase) 3.5.1.23 (Farber) + 1.3.1.35 +
GDP+Pi
CH3(CH2)14COCHCH2OH
NH3

CH3(CH2)14CH(OH)CHCH2OH
NH3
3.1.4.4
+
NH3
CH3(CH2)12CH CHCH(OH)CHCH2O Galactose
CH3C(OH)CH2CH2OP.O.P
Diphospho- O- O-
OH
CO2 -OOCCH CH COSCoA
2 2
6.3.5.4
5.4.99.2 HO–CCOO- CH.COO-
-OOCCH CH CH CH(N H )COO-
+ NH CHCH2CH2COO-
H2NCH2CH2CH2CH2CH(N H3)COO-
+

Galactosyl ceramidase 3.2.1.46 (Krabbe) Dehydrosphinganin Sphinganin

CH3(CH2)12CH
4-Sphingenin
CH.CH(OH)CHCH2OH
Psychosine Mevalonate 1.1.1.41 SUCCINYL-CoA 5.1.99.1
4.1.3.21
CH2COO-
4.2.1.36
CH(OH)COO- -OOHCCH CH CH COCOO-
2 2 2 2.6.1.39
2 2 2 3
OHCCH2CH2CH2CH(N H3)COO-
+ +
CH2CH2CH2CH2CH(N H3)COO- 1.5.1.7
1.5.1.8
LYSINE
Hyperlysinaemia
1.1.1.102 2.4.1.23 Homocitrate Homo- 2-Oxoadipate 2-Aminoadipate 2-Aminoadipate Saccharopine
2.3.1.50
Acyl-CoA 3.5.1.23UDP-Galactose Acyl-CoA
+ 1.2.1.31 1.5.1.10 Saccharopine dehydrogenase (NADP, lysine-forming) 1.5.1.8
-OOCCOCH CH COO-
1.2.4.2 H2NCOCH2CH(N H3)COO- isocitrate 1.1.1.155
semialdehyde 1.5.1.9
2.3.1.32
Sphingomyelin phosphodiesterase 3.1.4.12 (Niemann-Pick) Serine NHAcyl
+
NHCOR NHCOR 4.1.1.33 2 2 +
R.CH(N H3)COO-
Asparagine N
COO-
N
COO-
N
COO-
H2NCH2CH2CH2CH2COCOO- CH3CONH(CH2)4COCOO- CH3CONH(CH2)4CH(N H3)COO-
+
Saccharopine dehydrogenase (NADP, glutamate-forming) 1.5.1.10
CH3(CH2)12CH CHCH(OH)CHCH2OPOCH2CH2N(CH3)3
O-
3.1.4.12
CH3(CH2)12CH CH.CH(OH)CHCH2OH CH3(CH2)12CH CHCHOHCHCH2O Galactose CH2 2-OXOGLUTARATE 2-AMINO ACID Piperideine-6-
1.5.99.3
Pipecolate
H
Piperideine-2- 2-Oxo-6-amino- 2-Oxo-6-acetyl Acetyllysine
SPHINGOMYELIN 2.7.8.3 Ceramide 3.2.1.46 Cerebroside carboxylate 1.5.1.1
hexanoate aminohexanoate 1.4.3.2
Glucosyl-ceramidase 3.2.1.45 (Gaucher) CH3C.CH2CH2OPP 1.5.1.21 carboxylate 2.6.1.65 4-Hydroxybutyrate dehydrogenase 1.1.1.61
Gangliosides UDP-Sugars
2.4.1.62
2.4.1.47
3.2.1.45 Isopentenyl-PP TRANSAMINATION 1.3.99.7
-OOCCH CH CH COSCoA
2 2 2

R.CO.COO-
Glutaryl-CoA
CH3 C 5 GABA transaminase deficiency
RESPIRATORY CHAIN CH3C CHCH2OPP 2-OXO ACID 1.2.1.16 +
(CH3)3N CH2CH(OH)CH2COO-
+
(CH3)3N (CH2)3COO-
+
(CH3)3N (CH2)3CHO
+
(CH3)3N(CH2)3CH(OH)CH(N H3)COO-
+ +
(CH3)3N (CH2)3CH2CH(N H3)COO-
+

C 40 Lycopene Phytoene 2.5.1.1 1.2.1.47

Dimethylallyl-PP NAD+ Carnitine 4-Trimethylamino- 4-Trimethylamino- N6-Trimethyl- 1.14.11.8 N6-Trimethyllysine 4-Aminobutyrate aminotransferase 2.6.1.19
hv C 40 2.6.1. -
1.14.11.1 4.1.2.-
NADH dehydrogenase (Ubiquinone) 1.6.5.3 Lumirhodopsin C 5 -OOCCH CH CHO
2 2 1.1.1.61 -OOCCH CH CH OH
butyrate butyraldehyde 3-OH-lysine 4.1.1.50
4.1.1.71
ß-CAROTENE 2.5.1.29 CH3 CH3 Succinic 2 2 2 S-Adenosylmethylthiopropylamine Aminobutyrate deaminase 4.1.1.15
4-Hydroxybutyrate Decarboxylated S-adenosylmethionine
Succinic dehydrogenase (Ubiquinone) 1.3.5.1 C 40 Metarhodopsin Rhodopsin 2.5.1.32 CH3C CHCH2CH2C CHCH2OPP + semialdehyde 2.6.1.19
1.13.11.21 CH3O
O
CH3
CH2OPP
Geranyl-PP NADH -OOCCH CH CH(N H )COO-
2 2 3

4.1.1.15
-OOCCH CH CH N H
2 2 2 3
+
1.2.1.19 H2NCH2CH2CH2(CHO)
H2NCH2CH2CH2CH2NH2 H2N(CH2)4NH(CH2)3NH2 H2N(CH2)3NH(CH2)4NH(CH2)3NH2
Hyperprolinaemia I
Ubiquinol-cytochrome c reductase 1.10.2.2
COO- Opsin CH3O n
Geranyl-geranyl-PP
C 20
C 10
OPP GLUTAMATE 4-Aminobutyrate 4-Aminobutyraldehyde Putrescine 2.5.1.16 Spermidine 2.5.1.22
Spermine
Retinoate O Coenzyme Q CH2 CH2 CH2 CH2 CH2 HOCH CH2 Proline dehydrogenase 1.5.99.8
HOCH CH2
CHO Ubiquinone 2.5.1.10
ELECTRON 1.4.1.13 H2C CH.COO- 1.2.1.26
5.2.1.3 CH.COO- CH2 CH.COO- CH2 C.COO-
Cytochrome c oxidase 1.9.3.1 trans-Retinal 11-cis-Retinal CH2OH TRANSFER 3.5.1.2 CH
1.2.1.36 6.3.1.2
N NH 1.14.11.2
N
N OHCCH2CH2COCOO- Hyperprolinaemia II
Light CHO O
Phytol SYSTEM - 1.5.1.12 1.5.99.8 H

Retinol esters 1.1.1.105 1.1.1.105 C 20 Farnesyl-PP

1.6.5.3 NO3 H2NCO.CH2CH2CH(N H3)COO-
+ Pyrolline-5-
1.5.1.2
PROLINE HYDROXYPROLINE 4-Hydroxy- 3.5.4.22
2 Oxoglutarate-4-
Pyrroline-5-carboxylate reductase 1.5.1.2
PORPHYRIAS CH3 C 15
1.6.99.2
Glutamine carboxylate pyrroline- semialdehyde
Enz- FMN
1.7.99.4 1.6.6.1-3 1.4.1.2 HOCH CH2
O
Vitamin K HO 1.5.1.2 CH CH.COO-
2-carboxylate OHCCOO-

2.3.1.76
CH2OH CH3 O
2.5.1.21 Ox Red
- + +
Glyoxylate UREA CYCLE
Porphobilinogen synthase deficiency 4.2.1.24 Vitamin E 1.7.7.1 H2NCONHCH2CH2CH2CH(N H3)COO- N -OOCCH(OH)CH CH(N H )COO- -OOCCH(OH)CH COCOO- CH3COCOO-
3.1.1.21 trans-Retinol 5.2.1.7
11-cis-Retinol CH2OH CH3
a-Tocopherol
21
20
22
23
26
NO2 6.3.5.5
CITRULLINE 3-Hydroxy- 2 3

4-Hydroxy-
2

4-Hydroxy- Pyruvate
(Vitamin A) Dark 12
17 24 25 27 ATP CO2 4.1.1.17 pyrroline- 1.5.1.12 2.6.1.23 4.1.3.16
Citrullinaemia
Acute intermittent porphyria (AIP)
11 13 16 1.6.5.3
1.6.99.2 1.6.6.4
2.1.3.3 6.3.4.5 5-carboxylate glutamate 2-oxoglutarate
9 15
+ H2N.CO.O P
Enz- Fe-S
14
1

N2 1.18.6.1 6.3.4.16
Argininosuccinate synthase 6.3.4.5
Porphobilinogen deaminase 4.3.1.8
2
3
8

7
Red Ox 1.19.6.1 NH4 Carbamyl-P Legend
4
5
6
ATP 2.7.2.11
3.5.1.20
HOOCCHCH2COO-
Carbohydrates Biosynthesis Degradation
H H H Squalene N Most characteristics of human Hyperammonaemia (Type II)
Congenital erythropoietic porphyria HO HO HO HO 5.4.99.7
C 30 ADP+Pi 1.6.5.3
2.3.1.35
CH2 CH2 H2NCNHCH2CH2CH2CH(N H3)COO-
+
anatomy and physiology are Degradation
H H 1.14.99.7
CH2(NH3 )COO-
+
1.3.5.1
Amino Acids Biosynthesis Or nithine carbamoyltransferase 2.1.3.3
Uroporphyrinogen III synthase 4.2.1.75 CHOLESTEROL Desmosterol Zymosterol Lanosterol CHO CH.COO- Arginino- determined by biochemical reactions
COO- COO -
Glycine Ox Red P OOCCH2CH2CH(N H3)COO-
+
H2NCH2CH2CH2CH(N H3)COO-
+
3.5.3.6 Lipids Biosynthesis Degradation
COO- 2.3.1.37 Ubiquinone H2N succinate catalysed by thousands of specific
CH2 CH2 CH2
COO-
CH2
COO- Glutamyl-P1.2.1.41 Glutamic semialdehyde 2.6.1.13
ORNITHINE 1.14.13.39
Purines & Pyrimidines Biosynthesis Degradation enzymes. These, in turn, are Hyperammonaemia (Type I)
Porphyria cutanea tarda (PCT) CH H
CH3 CH H2
CH3 CH2 H2
CH3
-OOC CH2 H2
CH2
5.4.3.8 4.3.2.1
CH2 CH2 CH2 CH2 H2NCONH2 NO Pentose Phosphate Pathway determined by our genetic make-up, Carbamoylphosphate synthase (ammonia) 6.3.4.16
Uroporphyrinogen decarboxylase 4.1.1.37 H3C
C
CH H3C
C
CH H3C
C
CH2 H2C
C
CH2 ATP 1.10.2.2
ADP+Pi NHCOCH3 NHCOCH3 NHCOCH3 UREA
Photosynthesis
and genes are inherited – or inborn. If
N N N N N N N N 3.5.1.16
H H H H H H -OOCCH CH COCH NH +
-OOCCH CH CH.COO-
2 2 OHCCH2CH2CHCOO- H2NCH2CH2CH2CHCOO- Human Metabolism Vitamins, Co-enzymes, Hormones a gene is defective or missing it may
HC Fe CH H2C CH2 H2C CH2 H2C CH2 2 2 2 3
COO-
2.7.2.8 2.6.1.11 Argininosuccinic aciduria
Hereditary coproporphyria (HCP) H H H H H H 5-Aminolevulinate 2H+ Red Cytochrome-c Ox N-Acetylglutamate 1.2.1.38 N-Acetylglutamic N-Acetylornithine 3.5.3.1 NH2
+
Small numbers refer to Enzyme Commission result in defective or missing enzyme.
N N N N N N N N -OOC CH2 semialdehyde H2NCNHCH2CH2CH2CH(N H3)COO-
+
Reference Numbers for Enzymes This may well be lethal, but in some Argininosuccinate lyase 4.3.2.1
Coproporphyrinogen oxidase 1.3.3.3 H3C CH3 H3C CH3 H3C CH3 H2C CH3
C
H
C
H2
C
H2 -OOC
C
H2 COO-
H2C CH2
NH CO
O NH2
+
ARGININE cases it can lead to a Metabolic
ADP+Pi ATP
4.2.1.24 +
CH2 CH2 CH2 CH2 CH2 CH2 CH2 CH2
1.9.3.1 HN C -O P NHCN(CH3)CH2COO-
NH2 NH2
+
Deficiency Disease or Inborn Error of Hyperornithinaemia (Type I)
Variegate Porphyria (VP) CH2 CH2 CH2 CH2 CH2 CH2 CH2 CH2 H2C N Cytochrome N(CH3)CH2 H2NCN(CH3)CH2COO- H2NCNHCH2COO- +
H O- CH2(N H3)COO- Metabolism. For example, in the metabolic pathway a b c d, if the enzyme which Ornithine-oxoacid aminotransferase 2.6.1.13
COO- COO- COO- COO- COO- COO- COO- COO- 3.5.2.10
5-Aminolevulinate synthase 2.3.1.37 4.3.1.8 H2N oxidase Creatinine P-creatine Creatine Glycocyamine catalyses the reaction b c is defective, c may not be formed. The reaction sequence may,
Glycine
Protoporphyrinogen oxidase 1.3.3.4
HEME 1.3.3.4
Protoporphyrinogen
1.3.3.3
Coproporphyrinogen
4.1.1.37
Uroporphyrinogen
4.2.1.75
Porphobilinogen 2.7.3.2
3.9.1.1
2.1.1.2
Guanidoacetate
2.1.4.1
however, continue to produce b which can then concentrate in body fluids such as blood or
4.99.1.1 IX III III 1/2 O2 H 2O 3.5.2.10
urine. The knowledge of the site of the defective enzyme and of its corresponding gene(s) may
Hyperargininaemia
well make possible the rational treatment of the resulting disease. The Inborn Errors of Arginase 3.5.3.1
Erythropoietic Protoporphyria
Electron Flow from Reduced NAD and Succinate to Oxygen Metabolism Map names about 120 such diseases and identifies their sites of origin.
Ferrochelatase 4.99.1.1 Biochemical understanding thus provides an insight into clinical understanding.

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
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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
1.1.1.1 Alcohol dehydrogenase
1.14.13.9 Kynurenine 3-monooxygenase 2.7.2.6 Formate kinase 4.1.1.53 Phenylalanine decarboxylase
1.1.1.8 Glycerol-3-phosphate dehydrogenase
1.14.13.12 Benzoate 4-monooxygenase 2.7.2.11 Glutamate 5-kinase 4.1.1.65 Phosphatidylserine decarboxylase
1.1.1.9 D-Xylulose reductase
1.14.13.39 Nitric oxide synthase 2.7.3.2 Creatine kinase 4.1.1.71 2-Oxoglutarate decarboxylase
1.1.1.10 L-Xylulose reductase
1.14.16.1 Phenylalanine 4-monooxygenase 2.7.4.2 Phosphomevalonate kinase 4.1.2.5 Threonine aldolase
1.1.1.14 L-Iditol dehydrogenase
1.14.16.2 Tyrosine 3-monooxygenase 2.7.4.3 Adenylate kinase 4.1.2.9 Phosphoketolase
1.1.1.15 D-Iditol dehydrogenase
1.14.16.4 Tryptophan 5-monooxygenase 2.7.4.4 Nucleoside-phosphate kinase 4.1.2.12 Ketopantoaldolase
1.1.1.19 Glucuronate reductase
1.14.17.1 Dopamine ß-monooxygenase 2.7.4.6 Nucleoside-diphosphate kinase 4.1.2.13 Fructose-bisphosphate aldolase
1.1.1.21 Aldehyde reductase
1.14.18.1 Monophenol monooxygenase 2.7.4.9 dTMP kinase 4.1.2.14 2-Dehydro-3-deoxyphosphogluconate aldolase
1.1.1.22 UDPglucose dehydrogenase
1 14.99.1 Prostaglandin synthase 2.7.4.14 Cytidylate kinase 4.1.2.15 2-Dehydro-3-deoxyphosphoheptonate aldolase
1.1.1.23 Histidinol dehydrogenase
1 14.99.5 Stearoyl-CoA desaturase 2.7.6.1 Ribose-phosphate pyrophosphokinase 4.1.3.1 Isocitrate lyase
1.1.1.25 Shikimate dehydrogenase
1.14.99.7 Squalene monooxygenase 2.7.7.3 Pantetheine-phosphate adenylyltransferase 4.1.3.2 Malate synthase
1.1.1.27 Lactate dehydrogenase
1.14.99.25 Linoleoyl-CoA desaturase 2.7.7.4 Sulfate adenylyltransferase 4.1.3.4 Hydroxymethylglutaryl-CoA lyase
1.1.1.28 D-Lactate dehydrogenase
1.17.4.1 Ribonucleoside-diphosphate reductase 2.7.7.6 RNA nucleotidyltransferase (DNA-directed) 4.1.3.5 Hydroxymethylglutaryl-CoA synthase
1.1.1.29 Glycerate dehydrogenase
1.18.6.1 Nitrogenase 2.7.7.7 DNA nucleotidyltransferase (DNA-directed) 4.1.3.7 Citrate (si)-synthase
1.1.1.30 3-Hydroxybutyrate dehydrogenase
2.7.7.9 UTP—glucose-1-phosphate uridylyltransferase 4.1.3.8 ATP citrate (pro-S)-lyase
1.1.1.31 3-Hydroxyisobutyrate dehydrogenase TRANSFERASES 2.7.7.10 UTP—hexose -1-phosphate uridylyltransferase 4.1.3.16 4-Hydroxy-2-oxoglutarate aldolase
1.1.1.32 Mevaldate reductase
2.1.1.1 Nicotinamide N-methyltransferase 2.7.7.12 UDP glucose—hexose-1-phosphate uridylyltransferase 4.1.3.18 Acetolactate synthase
1.1.1.34 Hydroxymethylglutaryl-CoA reductase (NADPH)
2.1.1.2 Guanidinoacetate N-methyltransferase 2.7.7.13 Mannose-1-phosphate guanylyltransferase 4.1.3.20 N-Acylneuraminate-9-phosphate synthase
1.1.1.35 3-Hydroxyacyl-CoA dehydrogenase
2.1.1.3 Thetin—homocysteine S-methyltransferase 2.7.7.14 Ethanolamine-phosphate cytidylyltransferase 4.1.3.21 Homocitrate synthase
1.1.1.37 Malate dehydrogenase
2.1.1.4 Acetylserotonin N-methyltransferase 2.7.7.15 Choline-phosphate cytidylyltransferase 4.1.3.22 Citramalate lyase
1.1.1.38 Malate dehydrogenase (oxaloacetate-decarboxylating)
2.1.1.5 Betaine—homocysteine S-methyltransferase 2.7.7.18 Nicotinate-mononucleotide adenylyltransferase 4.1.3.27 Anthranilate synthase
1.1.1.39 Malate dehydrogenase (decarboxylating)
2.1.1.6 Catechol O-methyl transferase 2.7.7.22 Mannose-1-phosphate guanylyltransferase (GDP) 4.1.99.1 Tryptophanase
1.1.1.41 Isocitrate dehydrogenase (NAD+)
2.1.1.10 Homocysteine S-methyltransferase 2.7.7.23 UDP-N-acetylglucosamine pyrophosphorylase 4.2.1.2 Fumarate hydratase
1.1.1.43 Phosphogluconate dehydrogenase
2.1.1.13 5-Methyltetrahydrofolate—homocysteine 2.7.7.24 Glucose-1-phosphate thymidylyltransferase 4.2.1.3 Aconitate hydratase
1.1.1.44 Phosphogluconate dehydrogenase (decarboxylating)
S-methyl transferase 2.7.7.27 Glucose-1-phosphate adenylyltransferase 4.2.1.4 Citrate dehydratase
1.1.1.45 L-Gulonate dehydrogenase
2.1.1.14 5-Methyltetrahydropteroyltriglutamate — 2.7.7.34 Glucose-1-phosphate guanylyltransferase 4.2.1.9 Dihydroxy-acid dehydratase
1.1.1.49 Glucose-6-phosphate dehydrogenase homocysteine S-methyltransferase 2.7.7.41 Phosphatidate cytidylyltransferase 4.2.1.10 3-Dehydroxyquinate dehydratase
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 dehydratase
1.1.1.61 4-Hydroxybutyrate dehydrogenase 2.1.1.43 Histone-lysine N-methyltransferase 2.7.8.3 Ceramide cholinephosphotransferase 4.2.1.16 Threonine dehydratase
1.1.1.79 Glyoxylate reductase (NADP+) 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 2.7.8.8 CDPdiacylglycerol—serine O-phosphatidyltransferase 4.2.1.18 Methylglutaconyl-CoA hydratase
1.1.1.82 Malate dehydrogenase (NADP+) 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 Phosphoribosylaminoimidazole carboxamide 2.7.8.13 Phospho-N-acetylmuramoyl-pentapeptide-transferase 4.2.1.20 Tryptophan synthase
1.1.1.86 Ketol-acid reductoisomerase formyltransferase 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 HYDROLASES 4.2.1.36 Homoaconitate hydratase
1.1.1.155 Homoisocitrate dehydrogenase 2.1.3.3 Ornithine carbamoyltransferase
3.1.1.3 Triacylglycerol lipase 4.2 1.46 dTDPglucose 4,6-dehydratase
1.1.1.157 3-Hydroxybutyryl-CoA dehydrogenase 2.1.4.1 Glycine amidinotransferase
3.1.1.4 Phospholipase A2 4.2.1.47 GDPmannose 4,6-dehydratase
1.1.1.158 UDP-N-acetylmuramate dehydrogenase 2.2.1.1 Transketolase
3.1.1.5 Lysophospholipase 4.2.1.49 Urocanate hydratase
1.1.1.169 2-Dehydropantoate 2-reductase 2.2.1.2 Transaldolase
3.1.1.7 Acetylcholinesterase 4.2.1.51 Prephenate dehydratase
1.1.1.204 Xanthine dehydrogenase 2.3.1.1 Amino-acid N-acetyltransferase
3.1.1.17 Gluconolactonase 4.2.1.52 Dihydrodipicolinate synthase
1.1.1.205 IMP dehydrogenase 2.3.1.4 Glucosamine-phosphate N-acetyltransferase
3.1.1.21 Retinyl-palmitate esterase 4.2.1.55 3-Hydroxybutyryl-CoA dehydratase
1.1.3.8 L-Gulonolactone oxidase 2.3.1.5 Arylamine N-acetyltransferase
3.1 1.24 3-Oxoadipate enol-lactonase 4.2.1.58 Crotonoyl-[acyl-carrier-protein] hydratase
1.1.3.15 (S)-2-Hydroxyacid oxidase 2.3.1.6 Choline O-acetyltransferase
3.1.1.28 Acylcarnitine hydrolase 4.2.1.59 3-Hydroxyoctanoyl-[acyl-carrier protein] dehydratase
1.1.3.22 Xanthine oxidase 2.3.1.7 Carnitine O-acetyltransferase
3.1.1.31 6-Phosphogluconolactonase 4.2.1.60 3-Hydroxydecanoyl-[acyl-carrier protein] dehydratase
1.1.99.1 Choline dehydrogenase 2.3.1.8 Phosphate acetyltransferase
3.1.1.32 Phospholipase A1 4.2.1.61 3-Hydroxypalmitoyl-[acyl-carrier protein] dehydratase
1.1.99.5 Glycerol-3-phosphate dehydrogenase 2.3.1.9 Acetyl-CoA C-acetyltransferase
3.1.2.1 Acetyl-CoA hydrolase 4.2.1.75 Uroporphyrinogen-III synthase
1.2.1.3 Aldehyde dehydrogenase (NAD+) 2.3.1.12 Dihydrolipoamide S-acetyltransferase
3.1.2.3 Succinyl-CoA hydrolase 4.2.1.80 2-Oxopent-4-enoate hydratase
1.2.1.7 Benzaldehyde dehydrogenase (NADP+) 2.3.1.15 Glycerol-3-phosphate O-acyltransferase
3.1.2.4 3-Hydroxyisobutyryl-CoA hydrolase 4.2.99.2 Threonine synthase
1.2.1.8 Betaine-aldehyde dehydrogenase 2.3.1.16 Acetyl-CoA C-acyltransferase
3.1.2.11 Acetoacetyl-CoA hydrolase 4.2.99.8 Cysteine synthase
1.2.1.11 Aspartate-semialdehyde dehydrogenase 2.3.1.20 Diacylglycerol O-acyltransferase
3.1.3.2 Acid phosphatase 4.2.99.9 O-Succinylhomoserine (thiol)-lyase
1.2.1.12 Glyceraldehyde-3-phosphate dehydrogenase 2.3.1.21 Carnitine O-palmitoyltransferase
3.1.3.3 Phosphoserine phosphatase 4.3.1.1 Aspartate ammonia-lyase
1.2.1.13 Glyceraldehyde-3-phosphate dehydrogenase 2.3.1.23 Lysolecithin acyltransferase
3.1.3.4 Phosphatidate phosphatase 4.3.1.2 Methylaspartate ammonia-lyase
1.2.1.16 Succinate-semialdehyde dehydrogenase (NAD(P)+) 2.3.1.24 Sphingosine N-acyltransferase
3.1.3.5 5´-Nucleotidase 4.3.1.3 Histidine ammonia-lyase
1.2.1.18 Malonate semialdehyde dehydrogenase (acetylating) 2.3.1.30 Serine O-acetyltransferase
3.1.3.9 Glucose-6-phosphatase 4.3.1.4 Formiminotetrahydrofolate cyclodeaminase
1.2.1.19 Aminobutyraldehyde dehydrogenase 2.3.1.32 Lysine N-acetyltransferase
3.1.3.11 Fructose-bisphosphatase 4.3.I.8 Hydroxymethylbilane synthase
1.2.1.21 Glycolaldehyde dehydrogenase 2.3.1.35 Glutamate N-acetyltransferase
3.1.3.15 Histidinol-phosphatase 4.3.2.1 Argininosuccinate lyase
1.2.1.23 2-Oxoaldehyde dehydrogenase (NAD+) 2.3.1.37 5-Aminolevulinate synthase
3.1.3.25 myo-Inositol-1(or 4)-monophosphatase 4.3.2.2 Adenylosuccinate lyase
1.2.1.24 Succinate-semialdehyde dehydrogenase 2.3.1.38 [Acyl-carrier-protein] S-acetyltransferase
3.1.3.27 Phosphatidylglycerophosphatase 4.4.1.1 Cystathionine g-lyase
1.2.1.25 2-Oxoisovalerate dehydrogenase (acylating) 2.3.1.39 [Acyl-carrier-protein] S-malonytransferase
3.1.3.29 N-Acylneuraminate-9-phosphatase 4.4.1.8 Cystathionine ß-lyase
1.2.1.26 2,5-Dioxovalerate dehydrogenase 2.3.1.41 3-Oxoacyl-[acyl-carrier-protein] synthase
3.1.3.31 Nucleotidase 4.6.1.1 Adenylate cyclase
1.2.1.27 Methylmalonate-semialdehyde dehydrogenase (acylating) 2.3.1.46 Homoserine O-succinyltransferase
3.1.4.2 Glycerophosphocholine phosphodiesterase 4.6.1.3 3-Dehydroquinate synthase
1.2.1.28 Benzaldehyde dehydrogenase (NAD+) 2.3.1.50 Serine C-palmitoyltransferase
3.1.4.3 Phospholipase C 4.6.1.4 Chorismate synthase
1.2.1.31 L-Aminoadipate-semialdehyde dehydrogenase 2.3.1.51 1-Acylglycerol-3-phosphate O-acyltransferase
3.1.4.4 Phospholipase D 4.99.1.1 Ferrochelatase
1.2.1.32 Aminomuconate-semialdehyde dehydrogenase 2.3.1.76 Retinol O-fatty-acyltransferase
3.1.4.10 1-Phosphatidylinositol phosphodiesterase
1.2.1.36 Retinal dehydrogenase 2.4.1.1 Phosphorylase ISOMERASES
3.1.4.12 Sphingomyelin phosphodiesterase
1.2.1.39 Phenylacetaldehyde dehydrogenase 2.4.1.4 Amylosucrase
3.2.1.3 Glucan 1,4 -a-glucosidase 5.1.3.1 Ribulose-phosphate 3-epimerase
1.2.1.41 Glutamate-5-semialdehyde dehydrogenase 2.4.1.9 Inulosucrase
3.2.1.21 ß-Glucosidase 5.1.3.2 UDPglucose 4-epimerase
1.2.1.52 Oxoglutarate dehydrogenase 2 4.1.11 Glycogen (starch) synthase
3.2.1.23 ß-Galactosidase 5.1.3.4 L-Ribulose-phosphate 4-epimerase
1.2.3.5 Glyoxylate oxidase 2.4.1.13 Sucrose synthase
3.2.1.26 ß-Fructofuranosidase 5.1.3.6 UDPglucuronate 4-epimerase
1.2.4.1 Pyruvate dehydrogenase (lipoamide) 2.4.1.17 Glucuronosyltransferase
3.2.1.45 Glucosylceramidase 5.1.3.7 UDP-N-acetylglucosamine 4-epimerase
1.2.4.2 Oxoglutarate dehydrogenase (lipoamide) 2.4.1.21 Starch synthase
3.2.1.46 Galactosylceramidase 5.1.3.12 UDPglucuronate 5´-epimerase
1.2.7.1 Pyruvate synthase 2.4.1.22 Lactose synthase
3.2.1.48 Sucrose a-glucosidase 5 1.3.13 dTDP-4-Dehydrorhamnose 3,5-epimerase
1.2.7.2 2-Oxobutyrate synthase 2.4.1.23 Sphingosine ß-galactosyltransferase
3.3.1.1 Adenosylhomocysteinase 5.1.3.14 UDP-N-acetylglucosamine 2-epimerase
1.3.1.1 Dihydrouracil dehydrogenase (NAD+) 2.4.1.29 Cellulose synthase (GDP-forming)
3.5.1.1 Asparaginase 5.1.99.1 Methylmalonyl-CoA epimerase
1.3.1.2 Dihydropyrimidine dehydrogenase (NADP+) 2.4.1.32 Glucomannan 4-ß-mannosyltransferase
3.5.1.2 Glutaminase 5.2.1.2 Maleylacetoacetate isomerase
1.3.1.6 Fumarate reductase (NADH) 2.4.1.41 Polypeptide N-acetylgalactosaminyltransferase
3.5.1.6 ß-Ureidopropionase 5.2.1.3 Retinal isomerase
1.3.1.8 Acyl-CoA dehydrogenase (NADP+) 2.4.1.62 Ganglioside galactosyltransferase
3.5.1.7 Ureidosuccinase 5.2.1.7 Retinol isomerase
1.3.1.9 Enoyl-[acyl-carrier-protein] reductase (NADH) 2.4.1.68 Glycoprotein 6-a-L-fucosyltransferase
3.5.1.8 Formylaspartate deformylase 5.3.1.1 Triose-phosphate isomerase
1.3.1.10 Enoyl-[acyl-carrier-protein] reductase (NADPH, B-specific) 2.4.1.69 Galactoside 2-a-L-fucosyltransferase
3.5.1.9 Aryl-formamidase 5.3.1.3 Arabinose isomerase
1.3.1.13 Prephenate dehydrogenase (NADP+) 2.4.1.101 a-1,3-Mannosyl-glycoprotein ß-1,2-N-acetylglucosaminyltransferase
3.5.1.16 Acetylornithine deacetylase 5.3.1.4 L-Arabinose isomerase
1.3.1.14 Orotate reductase (NADH) 2.4.2.1 Purine-nucleoside phosphorylase
3.5.1.18 Succinyl-diaminopimelate desuccinylase 5.3.1.5 Xylose isomerase
1.3.1.26 Dihydrodipicolinate reductase 2.4.2.2 Pyrimidine-nucleoside phosphorylase
3.5.1.19 Nicotinamidase 5.3.1.6 Ribose-5-phosphate isomerase
1.3.1.35 Phosphatidylcholine desaturase 2.4.2.4 Thymidine phosphorylase
3.5.1.20 Citrullinase 5.3.1.8 Mannose-6-phosphate isomerase
1.3.3.3 Coproporphyrinogen oxidase 2.4.2.8 Hypoxanthine phosphoribosyltransferase
3.5.1.22 Pantothenase 5.3.1.9 Glucose-6-phosphate isomerase
1.3.3.4 Protoporphyrinogen oxidase 2.4.2.9 Uracil phosphoribosyltransferase
3.5.1.23 Ceramidase 5.3.1.15 D-Lyxose ketol-isomerase
1.3.5.1 Succinate dehydrogenase (ubiquinone) 2.4.2.10 Orotate phosphoribosyltransferase
3.5.1.32 Hippurate hydrolase 5.3.1.16 N-(5´-Phospho-D-ribosylformimino)-5-amino-1-
1.3.99.1 Succinate dehydrogenase 2.4.2.11 Nicotinate phosphoribosyltransferase
3.5.2.2 Dihydropyrimidinase (5´´-phosphoribosyl)-4-imidazolecarboxamide isomerase
1.3.99.2 Butyryl-CoA dehydrogenase 2.4.2.14 Amidophosphoribosyltransferase
3.5.2.3 Dihydroorotase 5.3.3.2 Isopentenyl-diphosphate ∆-isomerase
1.3.99.3 Acyl-CoA dehydrogenase 2.4.2.15 Guanosine phosphorylase
3.5.2.5 Allantoinase 5.3.3.4 Muconolactone ∆-isomerase
1.3.99.7 Glutaryl-CoA dehydrogenase 2.4.2.17 ATP phosphoribosyltransferase
3.5.2.7 Imidazolonepropionase 5.3.99.3 Prostaglandin-E synthase
1.3.99.10 Isovaleryl-CoA dehydrogenase 2.4.2.18 Anthranilate phosphoribosyltransferase
3.5.2.10 Creatininase 5.3.99.5 Thromboxane-A synthase
1.4.1.1 Alanine dehydrogenase 2.4.2.19 Nicotinate-nucleotide pyrophosphorylase (carboxylating)
3.5.3.1 Arginase 5.4.2.1 Phosphoglycerate mutase
1.4.1.2 Glutamate dehydrogenase 2.4.99.7 Sialyltransferase
3.5.3.4 Allantoicase 5.4.2.2 Phosphoglucomutase
1.4.1.7 Serine dehydrogenase 2.4.99.1-11 Sialyl transferases
3.5.3.5 Formiminoaspartate deiminase 5.4.2.3 Phosphoacetylglucosamine mutase
1.4.1.8 Valine dehydrogenase (NADP+) 2.5.1.1 Dimethylallyltransferase
3.5.3.6 Arginine deiminase 5.4.2.8 Phosphomannomutase
1.4.1.9 Leucine dehydrogenase 2.5.1.6 Methionine adenosyltransferase
3.5.4.1 Cytosine deaminase 5.4.3.8 Glutamate-1-semialdehyde 2,1-aminomutase
1.4.1.10 Glycine dehydrogenase 2.5.1.10 Geranyltranstransferase
3.5.4.3 Guanine deaminase 5.4.99.1 Methylaspartate mutase
1.4.1.13 Glutamate synthase (NADPH) 2.5.1.16 Aminopropyltransferase
3.5.4.6 AMP deaminase 5.4.99.2 Methylmalonyl-CoA mutase
1.4.1.14 Glutamate synthase (NADH) 2.5.1.19 3-Phosphoshikimate 1-carboxyvinyl-transferase
3.5.4.9 Methenyltetrahydrofolate cyclohydrolase 5.4.99.5 Chorismate mutase
1.4.1.19 Tryptophan dehydrogenase 2.5.1.21 Farnesyltransferase
3.5.4.10 IMP cyclohydrolase 5.4 99.7 Lanosterol synthase
1.4.3.1 D-Aspartate oxidase 2.5.1.22 Spermine synthase
3.5.4.12 dCMP deaminase 5.5.1.1 Muconate cycloisomerase
1.4.3.2 L-Amino-acid oxidase 2.5.1.29 Farnesyltranstransferase
3.5.4.19 Phosphoribosyl-AMP cyclohydrolase 5.5.1.4 myo-Inositol-l-phosphate synthase
1.4.3.4 Amine oxidase (flavin-containing) 2.5.1.32 Geranylgeranyl-diphosphate geranylgeranyl transferase
3.5.4.22 1-Pyrroline-4-hydroxy-2-carboxylate deaminase
1.4.3.6 Amine oxidase (copper-containing) 2.6.1.1 Aspartate transaminase
3.6.1.3 Adenosine-triphosphatase
LIGASES
1.4.3.8 Ethanolamine oxidase 2.6.1.2 Alanine transaminase
3.6.1.15 Nucleoside-triphosphatase 6.2.1.4 Succinate-CoA ligase (GDP-forming)
1.4.4.2 Glycine dehydrogenase (decarboxylating) 2.6.1.4 Glycine transaminase
3.6.1.31 Phosphoribosyl-ATP pyrophosphatase 6.2.1.25 Benzoate—CoA ligase
1.5.1.1 Pyrroline-2-carboxylate reductase 2.6.1.5 Tyrosine transaminase
3.7.1.2 Fumarylacetoacetase 6.3.1.1 Aspartate—ammonia ligase
1.5.1.2 Pyrroline-5-carboxylate reductase 2.6.1.6 Leucine transaminase
3.7.1.3 Kynureninase 6.3.1.2 Glutamate—ammonia ligase
1.5.1.3 Dihydrofolate reductase 2.6.1.9 Histidinol-phosphate transaminase
3.7.1.9 2-Hydroxymuconate-semialdehyde hydrolase 6.3.1.4 Aspartate—ammonia ligase (ADP-forming)
1.5.1.5 Methylenetetrahydrofolate reductase 2.6.1.11 Acetylornithine transaminase
3.9.1.1 Phosphoamidase 6.3.1.5 NAD+ synthetase
1.5.1.6 Formyltetrahydrofolate dehydrogenase 2.6.1.13 Ornithine—oxo-acid transaminase
6.3.2.1 Pantoate—ß-alanine ligase
1.5.1.7 Saccharopine dehydrogenase (NAD+), L-lysine-forming 2.6.1.16 Glutamine—fructose-6-phosphate transaminase LYASES 6.3.2.2 Glutamate—cysteine ligase
1.5.1.8 Saccharopine dehydrogenase (NADP+), L-lysine-forming 2.6.1.17 Succinyldiaminopimelate transaminase
4.1.1.1 Pyruvate decarboxylase 6.3.2.3 Glutathione synthase
1.5.1.9 Saccharopine dehydrogenase (NAD+), L-glutamate-forming 2.6.1.18 ß-Alanine—pyruvate transaminase
4.1.1.3 Oxaloacetate decarboxylase 6.3.2.5 Phosphopantothenate—cysteine ligase
1.5.1.10 Saccharopine dehydrogenase (NADP, L-glutamate-forming+) 2.6.1.19 4-Aminobutyrate transaminase
4.1.1.4 Acetoacetate decarboxylase 6.3.2.6 Phosphoribosylaminoimidazole-succinocarboxamide synthase
1.5.1.12 1-Pyrroline-5-carboxylate dehydrogenase 2.6.1.22 L-3-Aminoisobutyrate transaminase
4.1.1.9 Malonyl-CoA decarboxylase 6.3.2.7 UDP-N-Acetylmuramoyl-L-alanyl-D-glutamate—lysine ligase
1.5.1.21 ∆ -Piperideine-2-carboxylate reductase
1
2.6.1.23 4-Hydroxyglutamate transaminase
4.1.1.11 Aspartate 1-decarboxylase 6.3.2.8 UDP-N-acetylmuramate—alanine ligase
1.5.3.1 Sarcosine oxidase 2.6.1.27 Tryptophan transaminase
4.1.1.12 Aspartate 4-decarboxylase 6.3.2.9 UDP-N-acetylmuramoylalanine—D-glutamate ligase
1.5.99.1 Sarcosine dehydrogenase 2.6.1.32 Valine—3-methyl-2-oxovalerate transaminase
4.1.1.15 Glutamate decarboxylase 6.3.2.10 UDP-N-acetylmuramoylalanyl-D-glutamyl-lysine—
1.5.99.2 Dimethylglycine dehydrogenase 2.6.1.36 L-Lysine 6-transaminase
4.1.1.17 Ornithine decarboxylase D-alanyl-D-alanine ligase
1.5.99.3 L-Pipecolate dehydrogenase 2.6.1.39 2-Aminoadipate transaminase
4.1.1.20 Diaminopimelate decarboxylase 6.3.2.13 UDP-N-Acetylmuramoylalanyl-D-glutamate—2,6-diaminopimelate ligase
1.5.99.8 Proline dehydrogenase 2.6.1.42 Branched-chain-amino-acid transaminase
4 1.1.21 Phosphoribosylaminoimidazole carboxylase 6.3.3.1 Phosphoribosylglycinamidine cyclo-ligase
1.6.4.1 Cystine reductase (NADH) 2.6.1.51 Serine—pyruvate transaminase
4.1.1.22 Histidine decarboxylase 6.3.4.1 GMP synthase
1.6.5.3 NADH dehydrogenase (ubiquinone) 2.6.1.52 Phosphoserine transaminase
4.1.1.23 Orotidine-59-phosphate decarboxylase 6.3.4.2 CTP synthase
1.6.6.1 Nitrate reductase (NADH) 2.6.1.65 N6-Acetyl-b
b-lysine transaminase
4.1.1.25 Tyrosine decarboxylase 6.3.4.3 Formate—tetrahydrofolate ligase
1.6.6.2 Nitrate reductase [NAD(P)H] 2.6.1.66 Valine—pyruvate transaminase
4.1.1.28 Aromatic-L-amino-acid decarboxylase 6.3.4.4 Adenylosuccinate synthase
1.6.6.3 Nitrate reductase (NADPH) 2.7.1.1 Hexokinase
4.1.1.29 Sulfoalanine decarboxylase 6.3.4.5 Argininosuccinate synthase
1.6.6.4 Nitrite reductase [NAD(P)H] 2.7.1.2 Glucokinase
4.1.1.31 Phosphoenolpyruvate carboxylase 6.3.4.7 Ribose-5-phosphate—ammonia ligase
1.6.6.8 GMP reductase 2.7.1.3 Ketohexokinase
4.1.1.32 Phosphoenolpyruvate carboxykinase (GTP) 6.3.4.13 Phosphoribosylamine—glycine ligase
1.7.3.3 Urate oxidase 2.7.1.4 Fructokinase
4.1.1.33 Diphosphomevalonate decarboxylase 6.3.4.16 Carbamoyl-phosphate synthase (ammonia)
1.7.7.1 Ferredoxin—nitrate reductase 2.7.1.6 Galactokinase
4.1.1.34 Dehydro-L-gulonate decarboxylase 6.3.4.17 Formate—dihydrofolate ligase
1.7.99.4 Nitrate reductase 2.7.1.7 Mannokinase
4.1.1.36 Phosphopantothenoylcysteine decarboxylase 6.3.5.1 NAD+ synthetase (glutamine-hydrolysing)
1.8.1.3 Hypotaurine dehydrogenase 2.7.1.11 6-Phosphofructokinase
4.1.1.37 Uroporphyrinogen decarboxylase 6.3.5.2 GMP synthetase (glutamine-hydrolysing)
1.8.1.4 Dihydrolipoamide dehydrogenase 2.7.1.15 Ribokinase
4.1.1.38 Phosphoenolpyruvate carboxykinase (pyrophosphate) 6.3.5.3 Phosphoribosylformylglycinamidine synthetase
1.8.2.1 Sulfite dehydrogenase 2.7.1.16 Ribulokinase
4.1.1.39 Ribulose-bisphosphate carboxylase 6.3.5.4 Asparagine synthase (glutamine-hydrolysing)
1.8.3.1 Sulfite oxidase 2.7.1.17 Xylulokinase
4.1.1.41 Methylmalonyl-CoA decarboxylase 6.3.5.5 Carbamoyl-phosphate synthase (glutamine-hydrolysing)
1.8.7.1 Sulfite reductase (ferredoxin) 2.7.1.19 Phosphoribulokinase
4.1.1.43 Phenylpyruvate decarboxylase 6.4.1.1 Pyruvate carboxylase
1.8.99.1 Sulfite reductase 2.7.1.23 NAD+ kinase
4.1.1.45 Aminocarboxymuconate-semialdehyde decarboxylase 6.4.1.2 Acetyl-CoA carboxylase
1.9.3.1 Cytochrome-c oxidase 2.7.1.24 Dephospho-CoA kinase
4.1.1.47 Tartronate-semialdehyde synthase 6.4.1.3 Propionyl-CoA carboxylase
1.10.2.1 L-Ascorbate—cytochrome-b5 reductase 2.7.1.25 Adenylylsulfate kinase
4.1.1.48 Indole-3-glycerol-phosphate synthase 6.4.1.4 Methylcrotonoyl-CoA carboxylase
1.10.2.2 Ubiquinol—cytochrome-c reductase 2.7.1.28 Triokinase
1.10.3.3 L-Ascorbate oxidase 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 Order 1-800-325-3010 Quantity Pricing/Standing Orders
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1.13.11.21 ß-Carotene l5,15´-dioxygenase 2.7 1.47 D-Ribulokinase
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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 The
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