Inborn Errors of Metabolism - ERNDIM€¦ · 2h+ h+ oxaloacetate pyruvate succinyl-coa glutarate...
Transcript of Inborn Errors of Metabolism - ERNDIM€¦ · 2h+ h+ oxaloacetate pyruvate succinyl-coa glutarate...
2H+
H+
OXALOACETATE
PYRUVATE
SUCCINYL-CoA
GLUTARATE
CITRATE
MALATEOXO-2 - ASPARTATE
NO2-
NO3-
N2
NH4
CH3COSCoA
ACETYL-CoA
HOH
CO2
NAD
TRANSAMINATION
2H+
2H+
+
CO2
5-Amino-levulinate
Glycine
H+H+
H+H+
H+H+
c
H+ H+
F0
F1
F1
β2
1 αβ
α
γ
c-sub-units
AD P+ Pi
3.6.1.34
H+-tra
nsporting ATP synthase
α
δ
F6
oscp
ATP
+ PDA iP
FUMARATE
UQH2
UQ
UQH2
NADH+H+CH2COO-C(OH)COO-CH2COO-
CH(OH)COO-CHCOO-
2COO-
-OOCCOCH2CH2COO-
-OOCCH2CH2CO.SCoA
-OOCCOCH2COO-
CH3CH(OH)CH2CO.SCoA
-OOCCH=CHCOO-
Glyoxylate Cycle
-OOCCHO
GTP
GDPATP
+
HEME Protoporphyrinogen Coproporphyrinogen Uroporphyrinogen Porphobilinogen
5-Amino-levulinate
COO-CH2CH2
H2NCH2C=O
FADH2
FADCyt.b
Fe-S
SUCCINATEII
CH3COCOO-
PYRUVATE
Pi
X Y
Cyt.c1
Fe-S
2e-2e-
1e-
1e-
III
2UQ UQ
UQH22UQH2
2UQ._ UQ.
_
Cyt.bL Cyt.bH
Cyt.c
IV
Cu
A
Cu
BCu
A
2e-
Heme a
Heme a3
2H+
2H+4H+
4H+
2H+
NAD+
NADH+H+
NAD+
1.1.1.39
1.2.4.12.3.1.123.1.3.43
4.1.1.32
4.1.3.7
4.2.1.3
1.1.1.41
1.2.4.22.3.1.61
6.2.1.4-OOCCH2CH2COO-
1.3.5.1
4.2.1.2
1.1.1.37
6.4.1.1
4.1.3.8
4.1.3.1
4.1.3.2
ISOCITRATE
2.6.1.1 61.1.3.2
4.1.1.71
61.1.2.1
4.3.1.1
1.4.1.2 1.4.1.14
6.3.5.4
1.6.6.11.7.99.4
1.7.7.1 4.6.6.1 1.6.81.1
2.3.1.37
1.10.2.2
1.10.2.2
γ
εδ
β
33β
ATP
ADP
ENDERGONIC REACTION
3 .5.
6.1
I
FMNH2
FMN I
1.6. 5.3
2Fe -S (5 Clusters)
H+ H+H+ H+
ATP
4H+
2H+
2H+or
H+
PHOTO-SYSTEM
l
O2
3.6.1.34
NADP+
GlyceraldehydeRibulose-1,5-bis-P
2β
88
H+c
32
ε
a
32
α1
α
ε
α
β2
1
3
α
αβ
3
α
H+
H+
H+
H+
H+H+
H+
H+ H+H+
H+H+H+H+
H+
H+H+H+
H+H+
H+
H+
H+
H+
H+
H+H+
H+H+
H+H+
H+H+H+Translocated protons
H+
H+H+
H+Pi
Ferredoxin
4H+
PC PC
PCPQH2
2PQH2
PQ_.
2PQ_.Fe-S Cyt.f
PQ
2PQ
Cyt bf
2e-
1e- 1e-
2e-
2e-
2e-
2e-
Cyclic Photophosphorylation
PQ
PHOTO-SYSTEM II
Cyt bc
Non-cyclic electron flow
2e-2e-
Mn2e-
*
Protons from Water
H2O
H+
H+
2H+2H+
γCO2
Fixation
H+
ADPPi
H+
NADPH+H+
ATPTHYLAKOID MEMBRANE
CHLOROPLAST OUTER MEMBRANE
THYLAKOID LUMEN
STROMA
(ele
c
tric current)
P680Chl.a
QA
QB
Pheophytin
P700
Chl.A0
A1
Fe-S
ATP
ADP
ATP
1/2O2
H2O
PQH2
To Brain -
AMINO
ACIDS
PYRI
MIDINES
PURINES
CATECHOLAMINES
AROMATIC
AMINO
ACIDS
hvVISION
LIPID
PHOTOSYNTHESIS
PORPHYRINS
STEROIDS
ISOPRENOIDS
PHOSPHOLIPIDS
DEGRADATION
LIPID
BIOSYNTHESIS
PENTOSES
HEXOSES
POLYSACCHARIDES
COCH CH(NH )COO2 3+
FOLICACID
C1POOL
VALINE
ATP
ATP
4.1.1.4 1.1.1.30
CH COCH3 3
CH COCH COO3 2
CH CH(OH)CH COO3 23-OH-ButyrateAcetone
Acetoacetate
KETONE BODIES
HYALURONIC ACID DERMATAN BLOOD GROUPSUBSTANCESPEPTIDO-
GLYCAN
N-Ac-Neuraminate(Sialate)
UDP-Iduronate
UDP-N-Ac-Galactosamine UDP-
Galacturonate
GDP-Fucose
TDP-Rhamnose
ADP-Glucose
UDP-Glucose
TDP-4-Oxo-6-deoxyglucose
MANNOSE
CHITIN CHONDROITIN PECTIN
ALGINATES
INULIN CELLULOSE
O-ANTIGENS STARCH GLYCOGEN
LACTOSE
GALACTOSE
O
OH
HO
OH
OH
CH3 O
OPPTO
OH
OH
O
OHACNH
HO OH
O
OHHO OPPU
NHAC
OH
OH OH
OH
HOOH
NHCOCH
OH HO
O
OPPG
CH OH2
HO
P
UDP-N-Ac-Glucosamine
pyruvateN-Ac-Mannosamine-6-P UDP-Glucuronate
GDP-Mannose
N-Ac-Mannosamine UDP-N-Ac-Glucosamine N-Ac-Glucosamine-6-P
Sorbitol
Fructose-1-P
Erythrose-4-P
3-Dehydrogulonate
L-Xylose
L-Lyxose
D-Xylulose
D-Ribose
RibitolL-Arabitol L-Xylulose
L-Ribulose L-Ribulose-5-P
L-Xylulose-5-P
L-Arabinose Xylitol D-Xylose
D-Arabinose D-Ribulose
ASCORBATE
2, 3-Dioxogulonate
N-Ac-Glucosamine-1-P
Inositol-PInositolGlucuronateGulonate
Gulonolactone 2-Oxogulonolactone
Fructose
6-P-Gluconate
D-Ribulose-5-P
D-Xylulose-5-P
Mannose-1-P
GDP-Glucose
TDP-Glucose
UDP-Galactose
Galactose-P
Glucose-1-P
Glucose-6-P
Fructose-6-P
Fructose-1:6-bis-P
OH
H H HO
HOCH2 C C C C CO
OH OHH OH
H HO
HOCH2 C C C CO CO
OHH
H
OH H
C C
OH
CO
P-Ribosylamine
Glycerate2, 3-Diphospho-glycerate
3-P-Glyceraldehyde
1:3-bis-P-Glycerate
3-P-Glycerate
2-P-Glycerate
P-enolpyruvate
Inositol
CH OH2
CH OH2
HOCH
CH OH2
CH O2
HOCHP
Glycerol
UDP-GalactoseUDP-Sugars
CH CH OH3 2ETHANOL
Dihydroxy-acetone-P
(Glycerone-P)
Glucosamine-6-P
Phospho-serine
Chain elongation Mitochondrial
Serine
Serine
Indole-3-glycerol-P
3-Deoxy-D-arabino-heptulosonate-7-P
Indoleacetate(Auxin) Indoxyl
Formylkynurenine Kynurenine 3-Hydroxykynurenine
3-Hydroxyanthranilate
2-Amino-3-carboxymuconate semialdehyde
NH
OH
Tryptamine 1-(o-Carboxy phenylamino)1-deoxyribulose-5-P
N-(5-P-Ribosyl)anthranilate
Anthranilate
Catechol
NHIndole-
acetaldehyde
NH
CH CHO2
Indolepyruvate
NH
C-CH(OH)CH(OH)CH O2 PCH
2-Aminomuconate-6-semialdehyde
CH CH NH2 2 2
NH
1.2.3.7
4.1.1.43
3.5.1.9 3.7.1.3
4.2.1.20
1.13.11.6 4.1.1.45
2.4.2.18
4.6.1.4
4.1.1.48
1.14.13.9
4.6.1.3 Dehydro-quinate
4.2.1.10 1.1.1.25 2.5.1.19Shikimate-3-P Shikimate-5enolpyruvate 3-P
ChorismatePEP
2.7.1.71
91.1.4
.1
Homogentisate Phenylpyruvate
Cinnamate
Coumarate
Prephenate
2-Aminomuconate
Fumarate Aspartate
GLYCINE
Sarcosine
Hydroxy-pyruvate
P-Hydroxy-pyruvate
SERINE
Imidazoleglycerol-P
P-Ribosyl-ATP P-Ribosylformimino5-aminoimidazole-carboxamide-R P
Formiminoglutamate
Imidazolonepropionate
Urocanate
Cystathionine Homocysteine
Phosphoadenylyl-sulphate
(PAPS)
CH (NH )COOH2 3+
Histidinol-PHistidinolHistidinal3.1.3.15 2.6.1.91.1.1.23
3.6.1.315.3.1.162.4.2.17
Asparagine
ALANINE
3-Sulphinylpyruvate
Oxobutyrate
2-Aceto-2-hydroxy-butyrate
O-Phospho-homoserine
HO SCH CH NH2 2 2 2Hypotaurine
HO SCH CH NH3 2 2 2Taurine
Glutamate
γ-Glutamylcysteine
Glutathione
Glycine
Cysteine
ß-Alanine
Cysteate S-Adenosylhomocysteine
4.2.99.2
1.1.1.864.2.1.9
1.1.1.86 4.2.1.9 2.6.1.32
81.3.1.4
S-Adenosylmethionine
(SAM)
2-Methylaceto-acetyl-CoA
2-Acetolactate 2-3-Dihydroxyisovalerate
2-Oxo-isovalerate
3-Hydroxy-isobutyrate
3-Hydroxy-Isobutyryl-CoA
Methylacrylyl-CoA
2-Methyl-3-hydroxy-
butyryl-CoA
Tiglyl-CoA 2 Methylbutyryl-CoA
2:3-Di-OH-3-methylvalerate
2-Oxo-3-methylvalerate
Isobutyryl-CoA
Cysteinesulphinate
4.2.99.9
2-Isopropyl-malate
CH CH COCOO3 2
+CH CH(NH )COO3 3
CH CH(OH)COO3
HO SCH COCOO2 2
OCH COCOO2P
OCH CH(NH )COO2 3P
HOCH COCOO2
HOCH CH(NH )COO2 3+
+
OH
CH COCOO2
COO
OHOHO
COO
OHOH
HO P
COO
OHOH
O
CH2
P
COO
O-C-COOOH
OOH
OOC CH COCOO2
N H
HOC-CH(OH)CH(OH)CH 2OPCH
COO
NH2
COO
OHNH2COO
COO
OCH
OOCCH NHCH2 3
CH (CH ) COS-ACP3 2 14 CH (CH ) COSCoA3 2 14
CH (CH ) CH=CHCO-S-ACP3 2 2
CH CH=CHCO-S-ACP3 CH CH(OH)CH COS-ACP3 2
COSCoA
Linoleate
Oleoyl-CoA
Stearoyl-CoA Dehydrostearoyl-CoA OH-Stearoyl-CoA Oxostearoyl-CoA
Palmitoyl-ACP Palmitoyl-CoA
ACYL-ACP 2, 3-Enoyl-ACP
3, 4-Decenoyl-ACP
2, 3-Decenoyl-ACP
3-OH-Acyl-ACP 3-Oxoacyl-ACP
3-Oxo-Decanoyl-ACP
3-Oxo-Hexanoyl-ACP3-OH-Hexanoyl-ACP2, 3-Hexenoyl-ACP
Butanoyl-ACP Crotonoyl-ACP 3-OH-Butanoyl-ACP Acetoacetyl-ACP
Hexanoyl-ACP
3-OH-Decanoyl-ACPDecanoyl-ACP
Palmitoleoyl-ACP
γ-Linolenate Arachidonate Leukotriene B4
COSCoA
CO-S-ACP
Thromboxane B2
HOOCCH CO-S-ACP2Malonyl-ACP
HOOCCH CO-SCoA2Malonyl-Co-A
CH CO-S-ACP3Acetyl-ACP
CH O-CO-R2
CH OH2
R’-CO-OCH
FATTY ACIDACYL-CoA(Cytosol)
CH O-CO-R2
CH O2
R’-CO-OCHP
Cardiolipin Phosphatidylglycerol
AcetylcholineGlycerophosphocholine Lysolecithin
Cholineplasmalogen
CDP-choline Choline-P
Mevaldate
CH (CH ) CH=CHCH(OH)CHCH OH3 2 12 2
Dehydrosphingosine Sphingosine 4-Sphingenin Psychosine
Acyl-CoAAcyl-CoA
CerebrosideGalactoseCH (CH ) CH=CHCH(OH)CHCH O-3 2 12 2
NHCOR
Isopentenyl-PP(C5)
Dimethylallyl-PP(C5)
Geranyl-geranyl-PP(C20)
Farnesyl-PP(C15)
Squalene(C30)
Geranyl-PP(C10)
Desmosterol Zymosterol Lanosterol
CH C = CHCH O3 2 PP
CH3
Ceramide
GangliosidesNHCOR
CH O2 PP
OPPCH2
CHOLESTEROL
CH C(OH)CH CH OH3 2 2
CH COO2
CH C(OH)CH CHO3 2
CH COO2
NADP+Dehydroascorbate
OH
H OHOCH2 C C C C CO
OHH OH
OH
H OHOCH2 C C CO CO CO
H
NAD+Pi
ADPADP
Phytoene(C40)
Lycopene (C40)
ß-CAROTENE (C40)RhodopsinMetarhodopsin
Retinoatetrans-Retinal
11-cis-Retinoltrans-Retinol(Vitamin A)
Retinol esters
Dark
Light
Ubiquinone(Coenzyme Q)
MenaquinonePlastoquinone
Phylloquinone(Vitamin K)
OpsinCHO
CH OH2
CHO
CH OH2
CH3CH O3CH O3 n
O
O
O
O
Phytol (C20)
α-Tocopherol(Vitamin E)
Quinolinate
Quinolinate-nucleotide
Nicotinate-nucleotide
Desamino-NAD
5-Hydroxy-tryptophan
5-Hydroxytryptamine(SEROTONIN)
N-Acetyl-5-O-methyl-serotonin(MELATONIN)
NH
CH CH NHCOCH2 2 3HO
NH
CH CH NHCOCH2 2 3CH O3
+NAD( P)
NICOTINATE
N-Acetyl-serotonin
2.4.2.19
Dopamine Dopa
Dopaquinone
THYROXINEMELANIN
OHOH
CHOHCH NHCH2 3
OHOH
CHOHCH NH2 2
4-OH-3-Methoxy-phenylglycol
CHOHCH NH2 2
OHOCH3OCH3 CHOHCH OH2
OHOCH3
4-OH-3-Methoxy-D-mandelate
Cyclic AMP2H-folate
4H-folate
ATP
Dephospho-Coenzyme A
4-P-Pantetheine
4-P-Pantothenylcysteine
4-P-Pantothenate
Pantoate
6.3.2.1
1.1.1.169
3.5.1.22
2.7.1.33
NH
NH
O
O
O
O
CH OH2
OHOCH3
CH(OH)COO
H2
NH3+
CH-COOCO
O
COO
NRibose- PN+
COO
NH
CH COO2
O
OH
CH O2 PNHCOCH NH2 2
OHFormyl
glycinamide-R P
Urea
Formylglycinamidine-R P
Allantoate Allantoin URATE
ADP
Xanthine Hypoxanthine
Inosine
Adenosine
Adenylo-succinate
5-Aminoimidazole-R P
5-Amino-4-imidazolecarboxylate-R P
5-Amino-4-imidazole(N-succinylcarboxamide)-R P
5-Aminoimidazolecarboxamide-R P
Formylamido-imidazole-
carboxamide-R P
H NCONH2 2
HNH C2 CHO
NH RP
NH
C
CCO
CCO
OC NH
NH
HN
HN
H
CCO
CCH
OC NH
N
N
HN
Carbamoylß-alanine
Dihydro-uracil Uracil
d-ADP
d-ATP
GTP GDP
XANTHOSINE-P(XMP)
TTP
TDP
ß-Ureidoisobutyrate
Dihydrothymine
Thymine d-UMP d-CMP d-CDP
CDP
Carbamoylaspartate
Dihydroorotate
Orotate Orotidine-P Uridine-P(UMP)
UDP
3-Amino-isobutyrate
CHCNH2
CHOCNH
NCHCO
CHOCN H
HN
CH-CH3CO
OCNH
HNCH2
CH2CH2
CO
OCNH
HN
CO
CHOCNH
HN C CH3
Methylmalonylsemialdehyde
OHCCHCOOCH3
CH2CO
CH-COOOCNH
HN CHCO
C-COOOCNH
HN
H NCONHCH CH COO2 2 2
CC
CCH
RPHC
NH
NH OOC-CH-CH COO2
N
N
N
CHO
NH RP
NH
OCH C2
2.7.7.433.1.3.29
1.1.1.158
5.1.3.13
3.1.3.294.1.3.20
2.7.1.60
5.1.3.14
5.1.3.7
5.1.3.1
4.1.7.2
5.4.2.3
71.1.1.3
1.1.1.49
12.1.1.1
2.7.7.23
3.1.3.251.13.99.11.1.1.19
1.1.3.8
5.3.1.3
2.7.1.53
9.1.1.151.1.7.2
71.1.7.2
4.3.1.5
5.1.3.1
2.2.1.2
4.1.2.13
2.7.1.11
2.7.1.47
2.2.1.1
1.2.1.12
1.1.1.29
1.1.1.95
1.3.1.35
1.14.99.25
2.3.1.41
2.3.1.41
2.3.1.41
1.1.1.100
5.3.99.5
4.2.1.60
3.99.3.5
1.99.41.1
1.13.11.34
1.3.1.101.3.1.9
1.14.99.5
9.1.3.1
4.2.1.61
06.1.2.4
06.1.2.4
4.2.1.59
4.2.1.58
1.3.1.9
1.3.1.9
6.2.1.3 3.1.2.20
1.1.1.100
1.1.3.5
HOOC-COOHOxalate
Glycolate
HOCH CHO2Glycol
aldehyde
Ethanolamine-P
2.7.1.30
2.3.1.7
2.1.7.3
4.1.3.5
2.7.8.8
2.1.1.172.1.1.71
1.3.1.35 2.7.8.2
3.1.4.33.1.4.4
2.7.7.15
5.1.1.32.4.1.3
2.7.1.32
.1.7.2 28
1.2.4.12.3.1.121.8.1.4
2.1.6.2.1.4.1 1
1.1.1.4
2.3.1.50
1.1.1.102
3.8.7.2
26.1.4.2
3.2.1.46
5.2.1.31.2.1.36
2.3.1.763.1.1.21
5.2.1.7
5.4.99.71.14.99.7
4.2.1.75
1.3.3.3
4.99.1.1
2.5.1.29
4.1.1.28 2.3.1.5 2.1.1.4
6.3.5.16.3.1.5
11.2.4.22.7.7.18
2.6.1.5
1.2.1.32
1.13.11.5
2.1.1.28
2.1.2.2 6.3.3.1
3.5.2.5
01.1.4.1
1.7.3.3 1.1.3.22
4.1.1.28
4.1.1.21
2.1.2.3
2.4.2.1
1.5.99.2
2.1.1.5
2.6.1.22
3.5.2.3
2.6.1.511.4.1.7
3.1.3.3
2.6.1.52
2.1.3.2
ACETATE
Glycine
4.2.4.2
2.1.1.45
RP
CHCO
CHOC N
HN
1.3.1.14 2.4.2.10 4.1.1.23
7.7.7.27.7.7.2
2.7.7.7
.71.11.4
7.7.7.2
2.7.4.14
4.4.1.11.6.4.1
1.13.11.20
4.4.1.8
4.2.1.22
72.1.1.1
4.1.1.293.2.3.6
2.2.3.6
1.8.1.3
LACTATE
Acetaldehyde
2.7.1.24
2.7.7.3
4.1.1.36
Bile Acids
CH CH COSCoA3 2Propanoyl-CoA
13.1.1.1
2.1.3.14.1.1.415.1.99.1
6.3.2.5
2.1.1.142.1.1.13
1.1.6.4
CDP-Ethanolamine
1.1.1.35 4.2.1.17
+HO SCH CH(NH )COO2 2 3
3.5.2.7NHHN
CH
OOC CHCH CH COO2 2
CMP-N-Acetylneuraminate
CDP-diacylglycerol
CHOLINE
Ethanolamine
Glyoxylate
HISTAMINE
2.3.1.46Homoserine
Tyramine
Plant PigmentsTannins
Maleylacetoacetate
Fumarylacetoacetate
5.2.1.2
CH COO2
OH
OH
OH
CH CH NH2 2 2
OHOH
CH CH NH2 2 2
Hydroxyphenylpyruvate
α-Tocopherol(Vitamin E)
LIGNIN
d-GTP
OH
OH OH
O
HOOH
P
OPPT
CH3HO
OHOH
O
NADPH2.2.1.1
2.7.1.3
1.1.1.45
1.1.1.130
1.10.3.31.10.2.1
1.1.1.10
ATP
CH CH NH2 2 2
NH
HO
5.4.99.5
2.6.1.51.13.11.27
Indole
4.1.99.1
1.13.11.11
OHO
OH
NH C C CH CH O2 P
H HCOO
NH2
COO
6.3.5.3 6.3.2.6 4.3.2.2
GuanineDNA
6.3.4.44.3.2.2
CCO
CCH
HCNH
N
N
HN CCO
CCH
HCNH
N
N
HN
3.5.3.4
2.4.2 1.
2.7.7.62.7.7.6
6.7.7.2
6.7.7.2
2.7.4.62.7.4.32.7.4.4
2.7.4.6
2.4.2.15
1.1.1.205
6.3.4.16.3.5.2
2.7.4.4
3.4.5.3
2.2.2.3
2.4.2.1
.3.1.3 5
3.1.4.6
6.3.4.2
1.17.4.1
1.4.71.1
3.5.4.12
2.7.4.8
9.4.7.2
6.4.7.2
2.4.2.4
1.3.1.2
3.5.2.23.5.1.6
3.5.1.6 3.5.2.2 3.5.4.1
Diphospho-mevalonate
Mevalonate
3-Oxopentanoyl-CoA
3-Oxoacyl-CoA3-OH-Acyl-CoA2, 3-Enoyl-CoA
2, 3-Hexenoyl-CoA 3-OH-Hexanoyl-CoA
Acetoacetyl-CoA3-OH-Butanoyl-CoACrotonoyl-CoA
Pentanoyl-CoA 3-OH-Pentanoyl-CoA
1.1.1.35
1.1.1.35
1.1.1.157
1.3.99.3
1.3.99.2
1.1.1.35Odd C Fatty acids
Succinylhomoserine
CO2
2-OXO ACID
Glutamyl-P
Glutamine
UREA
P-CreatineCreatine Creatinine
Glycine
CITRULLINE
Glutamicsemialdehyde
2-AMINO ACID
Pyrroline-5-carboxylate
GlyoxylatePyruvate
4-Hydroxy-2-oxoglutarate
4-Hydroxy-glutamate
3-Hydroxy-pyrroline-
5-carboxylate
PutrescineH NCH CH CH CH NH2 2 2 2 2 2
Spermidine
Spermine
N -Trimethyllysine66N -Trimethyl-3-OH-lysine
Carnitine
Glutaryl-CoA Saccharopine2-Aminoadipatesemialdehyde
2-Aminoadipate2-Oxoadipate
Arginino-succinate
Guanidoacetate
Methylmalonyl-CoA
4.1.2.12
AspartylSemialdehyde
2, 3-Dihydro-dipicolinate
11.1.2.1
4.2.1.52 1.3.1.26 Piperideine-2, 6-dicarboxylate
N-Succinyl-2-amino-6-oxo-
pimelate
2.6.1.17N-Succinyl-2, 6diaminopimelate
Diamino-pimelate
3.5.1.18
3-Methyl-glutaconyl-CoA
(CH ) CHCH COSCoA3 2 2(CH ) CHCH COSCoA3 2 23-Methyl-
crotonyl-CoAIsovaleryl-CoA
OxopantoateHCHO
OOCCH C = CHCOSCoA2
CH3
CH COCHCOSCoA3
CH3CH CH=CHCOSCoA3
CH3CH CH(OH)CHCOSCoA3
CH3
C(OH)CH(OH)COOCH CH3 2
CH3
CH = CCOSCoA2
CH3HOCH CHCOS-CoA-2
CH3CH CHCO-SCoA3
CH3
C (OH)CH(OH)COOCH3
CH3
CH CH CHCOSCoA3 2
CH3
CH C = CHCOSCoA3
CH3
ATP
4-Aminobutyrate(GABA)
1.3.99.7
1.5.1.2
1.14.11.2
2.6.1.39 1.2.1.31
1.14.11.8
2.5.1.16
2.5.1.22
4.1.3.16
2.6.1.23
1.5.99.8
1.5.1.2
6.4.1.4
4.2.1.33
2.6.1.6
1.1.1.85 Oxoleucine
COOH
(CH ) CHCHCH(OH)COO3 2
S-Adenosylmethylthiopropylamine
(Decarboxylated SAM)
PROLINE
Coenzyme A
CH C= CHCH3 2
CH3
OCH
CH
3
3HO
CH3CH3
CH3
CH C-CH H O3 2 2C PPCH2
CH C= CHCH O2 2 PP
CH3
3-P-Glycerol
Phosphatidylethanolamine
CEPHALIN
21.4.1.3
Progesterone
1.5.1.12
2.7.3.2
4.2.1.173.1.2.4
2.6.1.32
51.1.4.4
Acetylserine 2.7.7.42.7.1.251.8.99.1Adenylylsulphate
(APS)
2.7.1.39
51.1.4.4
92.1.1.4
8.99.2.4
03.1.3.2
P-Ribosyl-PP
1.1.1.1
1.2.1.4
Aspartyl-P
7.1.1.5
2.7.1.40
81.3.1.4
.3.1.481
1.5.1.9
1.14.11.1
CHOLINE
1.1.1.2
3
1.3.1.2
NHCH CH SH2 2 NHCH CH CO2 2OCH C(CH ) CH(OH)CO2 3 2P
ADP- NHCH CH SH2 2 NHCH CH CO2 2OCH C(CH ) CH(OH)CO2 3 2
P-ADP- NHCH CH SH2 2 NHCH CH CO2 2OCH C(CH ) CH(OH)CO2 3 2
1.4.1.8
1.3.99.3
1.3.99.3
1.4.1.9
1.1.2.4 8
.1.1.402
1.3.99.7
1.8.99.2
+CH CO-OCH CH(NH )COO2 2 3
2.5.1.6
2.1.1.102.1.1.20
1.1.1.3
1.1.1.34.2.1.18
4.1.2.5
5.1.3.4
02.1.2.4
02.1.1.2
Glyoxylate
6.1.1.2
4.3.4.1
Hexanoyl-CoA
Butanoyl-CoA
6.3.5.5
11.2.7.2
NO1.14.13.39
3.5.3.63.5.3.1
4.1.1.17
2.7.7.41
4.1.6.2
1.1.1.100
1.1.1.8
15.1.3.2.1.3.2 51
2.7.8.5
SO -42
1.14.16.4
NH2 OOC OOC
52.1.1.4
1.3.1.13
1.3.1.13
2.7.4.6
CYTIDINE-triphosphate
(CTP)
Cytosine
2.4.2.9
4.1.1.11
2.6.1.18
4.3.1.3
4.2.1.49
3HSO-
1.1.1.105
2.3.1.41
NADPH
22.1.1.1
.1.3.2 4
5.5.1.4
3.2.1.26 3.2.1.48
2.6.1.161.1.1.14
2.7.7.34
4.2.1.47
2.4.1.682.4.1.69
2.4.1.9
5.3.1.8
2.4.1.11
O
OPPU
CH OH2
CH OH2
HO OH
OH
HO
2.4.1.21
25.1.2.4
1.2.1.18
81.1.2.4
OCH
C
CHOCN
HN
DP
CHCO
CHOC N
HN
RPPP
OCH2
H
C C
H
OHOHCHNHN
CH
CP
H
O
OCH2 C C
H
OH
H
OHC
H
C CCNH2
CCH
HCN
RP(PP)
N
N
N+
P
NH2H
O
OCH2 C C
H
OH
H
OHC
H
C COC
CCH
HCN
RP
N
N
NHPH
OCH2 C C
H
OHOHCO CH2 C
CONH2
CCH
HCN
RP
N
N
NHP
+OOCCH N(CH )2 3 3
Betaine
Betainealdehyde
2.4.1.16
5.1.3.6
2.4.1.33
2.7.7.13
5.4.2.8
5.3.1.8
O
OH
CH OH2
HO
O PHO
2.7.1.28
2.7.1.31
6.3.4.13
1.1.3.5
2.2.1.1
4.1.1.9
3.1.2.11
ß-OH-ß-Methyl-glutaryl-CoA
6.3.4.5
5.3.1.6
2.5.1.21
2.1.1.2
3.5.2.10
6.3.2.136.3.2.7-10
2.7.7.27
2.7.7.9
3.5.4.10
CCO
CCH
OC N RP
N
N H
HN
CCO
CCH
N
N
N
HN
RPH N2 C
2.7.4.6
1.17.4.1
2.7.4.6
2.6.1.13Carbamoyl-P
9.1.3.2
Carnitine
4.1.6.244.1.6.2
CH C(OH)CH COSCoA3 2
CH COO2
1.2.3.5
1.1.1.34
61.1.3.2
4.1.3.5
1.1.3.2 6
Menaquinone
1.1.1.4
5
4.2.1.16
Carnosine
CH CH NH2 2 2CNHN
C
HC
H
4.1.1.22
4.3.1.3
Ubiquinone
Plastoquinone
D-Ribose-5-P
NH
CH CH(NH )COO2 3HO+
O
OH
OH
HO
OPPU
COO-
O
OHOH
HO OPPU
COO-
OH
H OHHOCH2 C C CO
H
CO COO-
OCHOHCHOHAcNH
HO
OPC
OCO -CH OH2
OCHOHCHOHAcNH
HO
OH
COOCH OH2
O
OHOHHOHO
CH O2 P
O
OHACNH
HO OH
CH O2 P
O
OOHHOHO P
CH OH2
O
OHOH HOHO
CH OH2
O
OHHO OH
3
CH O2 PO
OHHO O
NHCOCH3
P
CH OH2CH OH2CH OH2
O
OHHO OH
NH2
CH O2 P
O
OH
HO
OHOPPU
CH OH2
O
O
HO
OH
OH
HO
P
CH OH2
CH OH2 O
OHO
OH
OH
O
OHOH
OH
CH OH2
O
OHHO
OH
OH
CH2CH OP2
OH
H H HOHC C C C
OH OHHCOO-HOCH2
H
OH OHOHC C C CHO
HH
HOCH2
H
OH OHHC C C CHO
HOH
HOCH2
OH
H OHHC C C CHO
HOH
HOCH2
H
OH HOHC C C CHO
OHH
HOCH2
OH
H HOH
C C C CHO
OHH
HOCH2
OH
H OHOHC C C CHO
HH
HOCH2
H
OH HOHC C C C
OHH H
OHCOO-POCH2
OH
H OHC C CO
H
POCH2 CH OH2
H
OH HC C CO
OH
HOCH2 CH OH2
CH OH2POCH2
H
OH OHC C CO
H
CH OH2POCH2
OH
H HC C CO
OH
CH OH2POCH2
H
OH OHC C CO
H
HOCH2 CH OH2
OH
H OHC C CO
H
HOCH2 CH OH2
OH
H HC C CO
OH
HOCH2 CH OH2
H
OH OHHC C C
HOH
HOCH2 CH OH2
OH
H OHOHC C C
HH
HOCH2 CH OH2
H
OH HOHC C C C
OHH H
OHCH OH2HOCH2
H
OH OHC C CHO
HPOCH2
H
OH HOHC C C CO
OHH
HOCH2 CH O2 P
H
OH OHOHC C C
HH
HOCH2 CH OH2
H
OH OHC C C
H
COH
HO
POCH2 CH OH2
P
H
OH OHC C C
H
COH
HOCH O2 POCH2
H
OH OHC C
H
C CHOOH
H
POCH2POCH2
O
OH
NH2
OH
CH O2 P
CHOHCHOPOCH2HOCH2COCH2OP
O
OHO OP P
OH
CH O2 PCHOHCOOPPOCH2
CHOHPOCH2 COO
COO
OH
OHOOH
COO
CH (CH ) CH(OH)CH COS-CoA3 2 14 2CH (CH ) CH(OH)CH COS3 2 14 2
CH (CH ) COCH COS-CoA3 2 14 2
CH (CH ) COCH COS-CoA3 2 14 2
CH (CH ) COS-CoA3 2 n+2
CH (CH ) CH=CHCOS-CoA3 2 14
CH (CH )3 2 nCH=CHCOS-CoA
CH (CH )3 2 5 2CH=CHCH COSACP
CH (CH )3 2 6CH=CHCOSACP
CH (CH )3 2 6COCH COSACP2CH (CH )3 2 6 2 2CH CH COSACP
CH (CH )3 2 2 2 2CH CH COSACP
CH CH CH COSACP3 2 2
CH (CH )3 2 6CH(OH)CH COSACP2
CH CH=CHCO.S-ACP3
CH (CH )3 2 2 2COCH COSACP
CH3COCH COSACP2
CH (CH )3 2 n 2COCH COSACPCH (CH )3 2 n 2CH(OH)CH COSACP
CH (CH )3 2 2 2CH(OH)CH COSACP
ACYL-CoA(Mitochondria)
CH (CH ) CH CH COSCoA3 2 2 2n
CH (CH ) CH=CHCOSCoA3 2 n CH (CH CH(OH)CH COSCoA3 2n
CH (CH ) CH CH COSCoA3 2 2 2 2 CH (CH ) CH=CHCOSCoA3 2 2
CH (CH ) COCH COSCoA3 2 2n
CH COCH COSCoA3 2
CH (CH ) CH(OH)CH COSCoA3 2 2 2
CH CH(OH)CH COSCoA3 2CH CH=CHCOSCoA3CH CH CH COSCoA3 2 2
CH CH CH CH COSCoA3 2 2 2Pentenoyl-CoACH CH CH=CHCOSCoA3 2 CH CH CH(OH)CH COSCoA3 2 2
CH (CH ) COCH COSCoA3 2 2 2
CH CH COCH COSCoA3 2 2
OH OH
HO OH
O
O-CH O-PO2
CH CHOHCH OH2 2
O
CH2
CH2
R'-CO-OCH
O-CO-R
O-POO-
HOCH CH2 2NH3+POCH CH2 2NH3
+CPP- OCH CH2 2NH3
+
O
-
CH O-CO-R2
CH O P2 OCH CH2 2NH3+
CH COCH CH N(CH )3 2 2 3 3
HOCH CH N(CH )2 2 3 3+
CH CH 2 COO(NH )3+
OHOH
CH CH 2 COO-(NH )3+
CONH2
CH CH(NH )COO2 3+
NHOCCO
NHOC
NH
CH
H N2
HC CHN
N
CRP
H N2
CHC
CHOC N
N
DP
NH2
RPPP
CHC
CHOC N
N
NH2
CC
CCH
RP(P)HC N
N
N
NNH2
H NCOO2 P
(CH ) NH2 3 2H N(CH ) NH2 2 3 (CH ) NH2 4
(CH ) NH2 3 2H N(CH ) NH2 2 4
CH -SCH CH CHNH3 2 2 2
Adenosyl
+
+CH3
CH3
CHCH(NH )COO3
++CNHN
C
HC
H
CH CH(NH )CH OH2 3 2+
CNHN
C
HC
H
CH CH(NH )CH OP2 3 2CNHN
CH
HC CH COCH OP2 2
+HSCH CH CH(NH )COO2 2 3
+
+SCH CH CH(NH )COO2 2 3
CH CH(NH )COO2 3
RP
CO
C-COOOCN
HN CH
2.4.1.23
CH (CH ) CH=CHCH(OH)CHCH OH3 2 12 2
NH3+
CH (CH ) CH(OH)CHCH OH3 2 14 2
NH3+
CH (CH ) COCHCH OH3 2 14 2
NH3+
GalactoseCH (CH ) CH=CHCH(OH)CHCH O-3 2 12 2
NH3+
4.1.1.70
H NCONH2 2
OHCCH N(CH )2 3 3+
C-CH3CO
CHOCN DP
HN
DP
CPP-OCH CH N(CH )2 2 3 3+
OCH CH N(CH )2 2 3 3+
1.2.1.41
UDP-N-Ac-Muramate
O
OCH CH3
COO-NHAC
HO OPPU
CH OH2
4.1.3.20
O
OH
CH3
HO HO OPPG
2.7.7.24
2.4.1.22
2.7.7.125.1.3.2
4.1.3.5
Sedoheptulose-PP
3.1.1.28
4.2.1.24
N
CH2
CH2
N
N
N
CH3
CH3
CH
CH
COO
CHCHCH2
H C3
H C3H C3
HC
C
C
H
H
Fe
CH2CH2COO- -
CH3
CH3
CH2
CH2
CH2
H C3
H C3NH
NH
NH
NH
CH
CH
H C2
C
CH2
H2
-
CH2CH2COO
CH2CH2COO -
H C2
H C2
CH 2CH 2
OOC
COO
H N2
NH
-
-
CH2
H C2
H C2
H C2
H2
H2
CH2CH2 CH2
CH2
CH2
CH2
NH
NH
NH
NH
COO
COO
COOCOO
C
C
--
CH2CH2COO
CH2CH2COO- -
-
-
OOC-
OOC-CH3
CH3
H C3
H C3
H C2
H2
H2
NH
NH
NH
NHCH2
CH2
CH2
CH2
CH2
C
C
COO-
CH2CH2COO
CH2CH2COO- -
COO-
COO-
CH C(OH)CH CH O3 2 2 PPCH COO2
OHCCOO
HOCH COO2
NH2H NCNHCH COO2 2
+ NH2
H NCN(CH )CH COO2 3 2
+ NH2HNCN(CH )CH COO3 2P-
+ HN CNH CO
N CH2
CH3
OOCCHCH COO2
H NCNHCH CH CH CH2 2 2 2
N(NH )3+
COO
CHCOOHCCH2HOCH
N
OOCCH(OH)CH CH(NH )COO2 3+
CHCOOCH2HOCH
H C2NH
NH
CHCOO
CH2CH2CH2
OOCCH(OH)CH COCOO2
NCHCOO
CH2CH2
CH
CH COCOO3 OHCCOO
+H NCONHCH CH CH CH2 2 2 2 (NH )3 COO
H NOCCH CH CH2 2 2 COO(NH )3+
R-CO-COO
+
COOR-CH(NH )3+
COOH NOCCH CH2 2 (NH3+
OOC-CH-COSCoA
CH3
P OOCCH CH2 COO(NH )3+
OOCCH CH CHO2 2
OHCCH CH2 COO(NH )3+
(CH ) NCH CH(OH)CH COO3 3 2 2+
OHCCH CH CH2 2+
COO(NH )3
POOCCH CH CH2 2+
COO(NH )3
OHCCH CH CH CH2 2 2 COO(NH )3+ NH CHCH CH COO2 2
COO
CH CH CH CH CH2 2 2 2 COO(NH )3+
H N(CH )2 42 CH(NH )COO3CH(NH3++
(CH ) N(CH )3 3 2 3CH CH(NH )COO2 3+ +
CH2CH
OOCCN
HCCH-COO
CH2CH2
CH-COO OOCCN
H C2
OOCCOCH CH CH CH-COO2 2 2CH CH2 2 2
OOCCH CH CONH2 2 OOCCHCH CH CH CH-COO2 2 2
OOCCH CH CONH2 2CH2 2
NH3+
OOCCH-CH CH CH CHCOO2 2 2NH3
+
NH3+
(CH ) CHC(OH)CH3 2 2
COOH
COO (CH ) CHCH COCOO3 2 2
+(CH ) CHCH3 2 2 CH(NH )COO3
HOCH CHCOO2
CH3
CHCOCOOCH3
CH3CH COC(OH)CH3 3
COO
+POCH CH CH(NH )COO2 2 3
+HOCH CH CH(NH )COO2 2 3
+CH CH CH(NH )COO2 2 3 OOCCH CH2COO2
+CH SH2
OOCCH(NH )CH CH CONHCHCONHCH COO3 2 2 2
HOCH C(CH ) COCOO2 3 2
HOCH C(CH ) CH(OH)COO2 3 2
NHCH CH COO2 2HOCH C(CH ) CH(OH)CO3 2)2 3 2
NHCH CH COO2 2OCH C(CH ) CH(OH)CO2 3 2P
CH CH3 2
CH3CH
+CH(NH )COO3
CH COC(OH)CH CH3 2 3
COO CHCOCOOCH CH3 2
CH3
NHCHCH SH2
COO NHCH CH CO2 2OCH C(CH ) CH(OH)CO2 3 2P
+ CH SH2 OOCCH(NH )CH CH CONHCHCOO3 2 2
+AdenosylSCH CH CH(NH )COO2 2 3
+SCH CH CH(NH )COO2 2 3
+HO SCH CH(NH )COO3 2 3
+
+S-CH CH(NH )COO2 3
S-CH CH(NH )COO2 3+HSCH CH(NH )COO2 3
1.1.1.23
H
CNHN
C
HC CH CHCOO2NHCOCH CH NH2 2 2
+
H
CNHN
C
HC CH CH(NH )COO2 3
CHCH CH COO2 2NHN
CH
OC C CH CHCOONHN
CH
CH
CH COO3
NH
OOC
CH-COOOCNH2 CH2
H NCH CH COO2 2 2
H NCH2 2
CH3CHCOO H NCONHCH CHCOO2 2
CH3
CCO
CHRPHC N
N
N
HNC
CCO
CHHCO
N
N
N RP
CCH
N
NH N2
HC
CCO
CHN
NCC
CHN
N
RPH N2
H N2C
O
OO P~ ~P O
O
O OP O
O
CH2
N
N
N
NO
OHOH
NH2
HCCHN
N
N
N O
OP O O
O
CH2
OH
NH2
HCCH
COOCO
NHOC
NH
CH
H N2NH2
OOC-CH-CH COO2
HNCO CCH
N
N
CRPRP
H N2
OOCC CH
N RP
N
CH N2
CH=CHCOO
OH
CH=CHCOO
CH COCOO2
CH2
P
COO
OHO
O
O
COO
O
CH COO2OCH COO2-OOC
OCH2 CH2
OHHC
P
HOCH HCOH
OCCOO
COOHO
OHOHO
NH2COOOCH
NH
CH COCOO2NH
CHOCONH2
OH
CH CH(NH )COO2 3+
N
COOCOO
N+
COOCOO
RP
O
OH
OH
HO OPPU
COO
O
OH
NHCOCH3
HO
OPPU
COOCH OH2
O
OCCH2 NHAC
HO OPPU
COO
CH OH2
O
OHHO OHOH
COO
OOCCH N(CH )2 3 2Dimethylglycine
1.1.99.1
1.2.1.8
COOHO HO COO
O
HO OH
COO
CH(O )PPOCH2 COO CH(OH)HOCH2 COOCOO
CH(O )PHOCH2 COO
CH =C(O )2 P COO
CONH2
Ribose -O - P - O - P - O- Adenosine(P)+N
O
O
O
O
+COO
NRibose - O - P - O - P - O -Adenosine
O
O
O
O
II
IIOH
O
CH CH 2 COO(NH )3+Epinephrine
(Adrenaline)Norepinephrine
(Noradrenaline)
Normetepinephrine(Normetadrenaline)
HOCH CH N(CH )2 2 3 3+
Adenosyl+
+SCH CH CH(NH )COO2 2 3CH3
(CH ) N(CH )3 3 2 3CH CH(NH )COO2 3+ +OH
OOCCH CH CH COSCoA2 2 2
OOCCH CH CH NH2 2 2 2
OOCCH CH CH COCOO2 2 2 OOCCH CH CH CH2 2 2 COOCOO(NH )3++
HOH
HOH
HHO
HHO
H
Prostaglandin PGE 2
OH
HOCH2 C C CO C COO
OHH
OHH H
-
COO
OR'-CO-OCHCH O-CO-R2
CH O POCMP2O
CH CH N(CH )2 2 3+
OHOCHCH OPO2
CH OH2
O-
CH OPO2
OCH OCH=CHR2
CH CH N(CH )2 2 3 3+
O- CH OPO2
OR'-CO-OCHCH O-CO-R2 3.1.1.32
CH CH N(CH )2 2 3 3+
O-
OHOCHCH OPO2
CH O-CO-R2
CH CH N(CH )2 2 3 3+
O-
CH CH(NH )COO2 3+
+C
NHNC
HC
H
CH CH(NH )CHO2 3
HS
11-cis-Retinal
Pi
ADP
NADP+
GDP-Mannuronate
Mannose-6-PDehydro-shikimate
Shikimate
RNA
d-GDP
d-CTP
GUANOSINE-P(GMP)
ß-Alanine
P-Ribosyl-AMP P-Ribulosylformimino5-aminoimidazole-carboxamide-R P
Imidazoleacetol-P
HS HSO-
PANTOTHENATE
3-Isopropyl-malate
Succinicsemialdehyde
2.4.1.17
Diacylglycerol
2.3.1.6
1.14.12.1
1.4.4.2
CH CHO3
3.5.4.19
4.2.1.19
3.3.1.1
4.1.1.12
4.1.2.5
COO-2OHCCH
2.1.4.1
2.4.99.7
5.1.3.12
1.1.1.132
4.2.1.462.7.1.7
2.4.1.292.4.1.21
2.4.1.13
5.4.2.2
2.7.1.6
2.7.7.10
3.1.1.18
4.1.1.34
2.7.1.47
2.7.1.16
ADP
P-Gluconolactone
1.1.1.44
O
OOHHO
OH
CH O2 P
PHENYLALANINETYROSINE
TRYPTOPHAN
INOSINE-P(IMP)
THYMIDINE-P
HISTIDINE
URIDINE-triphosphate
(UTP)
CYSTINE CYSTEINE
ISOLEUCINE
LEUCINE
LYSINE
ORNITHINE
ARGININEHYDROXYPROLINE
PHOSPHATIDYLSERINEPhosphatidyl
inositol
LECITHIN
SPHINGOMYELIN
STEROIDS
Pregnenolone
2.7.6.1
2.4.2.146.3.4.7
5.3.1.9
3.1.3.11
1.2.1.13
2.7.2.3
H
OH OHC C C
H
COOHC
H
H
OH
POCH2 CH O2 P
5.4.2.1
ADP
4.2.1.11
Endoplasmic Reticulum
1.1.1.100
3.1.1.3
Phosphatidate
2.3.1.39
4.2.1.17
4.2.1.17
4.2.1.55
1.1.1.79
1.2.1.21
1.4.3.8
1.1.1.32
2.7.1.362.7.4.2
4.1.1.33
2.5.1.1
2.5.1.10
2.7.8.5
2.7.8.1
4.1.1.65
2.7.7.14
1.3.99.7
POO
CH (CH ) CH=CHCH(OH)CHCH O3 2 12 2
NHAcylCH CH N(CH )2 2 3 3
+O-
3.5.1.23
1.13.11.21
2.5.1.32
1.1.1.105
2.4.2.19
1.2.1.32
METHIONINE
4.1.3.27
4.2.1.51
1.14.17.1
1.14.18.1
1.14.13.11
Glycinamide-ribosyl-P
4.2.1.22
3-Oxohexanoyl-CoA
THREONINE
1.5.1.7 - 10
GLUTAMATE
2.3.1.38
ATP
NADH
SUCROSE
ADENOSINE-P(AMP)
TriacylglycerolFAT
RNA
CH3
CH2
OC-COOOH
COO
CH CH 2 COO(NH )3+
GLYCOPROTEINSGANGLIOSIDES
MUCINS
4.1.2.-
2.6.1.-
GLUCOSE
O-Acyl-carnitine
O-Acyl-carnitine
OH
O-
-
R-CO-OCH
R'-CO-OCH
R'-CO-OCH
O HCO-CO-R CH CH(OH)CH O-P-OCH2 2 2
CH O-CO-R’2OR'-CO-OCH
O-
CH O-CO-R2
CH O-PO2O-
2.7.8.11
H NCH CH CH CH2 2 2 2 COO(NH )3+
2.3.1.20 3.1.3.4 2.7.1.107
NH2+
+H NCNHCH CH CH CH2 2 2 2 COO(NH )3
H
OH HOHC C C CO
OHH
HOCH2 CH OH2
R-CH2COO
CH O-CO-R2
CH O-CO-R"2
R’-CO-OCH
OH
O
CH CHNH2 3+
OR'-CO-OCH
O-
CH O-CO-R2
CH O PO2
COO
OOCCH CH CH2 2 COO(NH )3
+CH CH(OH)CH(NH )COO3 3
O
OHHO
OH
OH
CH OH2CH OH2
NH
CH O-CO-R2
1.3.1.13
4.1.1.28
Malonicsemi-
aldehyde
4.2.7.2
2.1.3.3
3.5.1.26.3.1.2
5.5.3.6
CarbohydratesBiosynthesisDegradation
Amino AcidsBiosynthesisDegradation
LipidsBiosynthesisDegradation
Purines &Pyrimidines
BiosynthesisDegradation
Biosynthesis DegradationVitamins Co-enzymes & Hormones
Pentose Phosphate Pathway
Photosynthesis Dark Reactions
COMPARTMENTATIONThe "Backbone" of metabolism involves
GLYCOLYSIS in the CYTOPLASM,the TCA CYCLE (mainly) in the Mitochondrial matrix
and ATP FORMATION spanning theMITOCHONDRIAL INNER MEMBRANE
An electron flow (an electric current) generated fromNADH and UQH2 drives the translocation of protons
from the matrix to the intermembrane space.The retrolocation of these protons through the F0 subunits
of ATP synthase to the matrix then supplies the energyneeded to form ATP from ADP and phosphate
Electron Flow Proton Flow
Small Numbers ( eg. 2.4.6.7) refer to the IUBMBEnzymeCommission (EC) Reference Numbers of Enzymes
LEGEND
Human Metabolism is identified as far possible by black arrowsBiosynthesis Degradation
HEMOGLOBIN CHLOROPHYLL
ATP
TRA
NSLO
CATED PROTONS
2.7.1.1
ATP2.7.1.2
3.7.1.2
2.4.1.1
3.2.1.33 3.2.1.23
3.2.1.108
2.7.1.6
2.7.7.12
3.1.3.9
3.2.1.102.4.1.25
2.4.2.8
3.5.4.4
1.5.1.3
1.4.4.2
2.6.1.44
4.1.3.4
1.9.3.1
4.3.1.84.1.1.371.3.3.4
1.3.99.3
3.2.1.45
3.2.1.52
3.1.4.12
1.14.16.1
1.14.18.1
1.17.3.2
4.2.1.22
6.4.1.3
1.2.1.24
2.6.1.19
6.3.4.16
2.1.3.3
4.3.2.1
6.3.4.5
5.4.99.2
1.3.99.10
1.2.4.4
1.2.4.4
1.2.4.4
© 2006 International Union of Biochemistry and Molecular Biology www.iubmb.org
Designed by Donald E.Nicholson,The University of Leeds,England – and Sigma-Aldrich in collaboration with Mick Henderson,St.James’ Hospital,Leeds – and the UK MetBioNet Training Group www/metbio.net.For further information about metabolic disorders visit the OMIM (Online Mendelian Inheritance in Main) website, hosted by the John Hopkins University – www.ncbi.nlm.gov/entrez/query/fcgi?db=OMIM.
GDS
Inborn Errors of Metabolism
AMINO ACID METABOLISM
Fumarylacetoacetase(3.7.1.2)Tyrosinaemia Type I MIM 276700
Phenylalanine hydroxylase(1.14.16.1)Phenylketonuria (PKU) MIM 261600
Ty rosinase(1.14.18.1)Tyrosine Negative AlbinismMIM 203100
Glycine dehydrogenase(1.4.4.2)Non KetoticHyperglycinaemia (NKH) MIM 238300
PURINE & PYRIMIDINEMETABOLISM
Xanthine oxidase(1.17.3.2)XanthinuriaMIM 278300
Hypoxanthine guanine phospho-ribosyl transferase(2.4.2.8)HGPRT Deficiencey, PrimaryGout, Lesch-Nyhan SyndromeMIM 308000
Adenosine deaminase(3.5.4.4)Severe Combined Immuno-Deficiency (SCID) MIM 102700
FOLIC ACID
Dihydrofolate reductase(1.5.1.3)Megaloblastic Anaemia MIM 126060
ORGANIC ACIDURIAS
Propionyl-CoA carboxylase(6.4.1.3)Propionic Acidaemia MIM 606054
Methylmalonyl-CoA mutase(5.4.99.2)Methylmalonic AcidaemiaMIM 251000
Isovaleryl-CoA dehydrogenase(1.3.99.10)Isovaleric Acidaemia MIM 243500
AMINO ACID METABOLISM
Cystathionine synthetase(4.2.1.22)HomocystinuriaMIM 236200
Branched chain ketoaciddecarboxylase(1.2.4.4)Maple Syrup Urine DiseaseMIM 248600
GABA METABOLISMNEUROTRANSMITTERDEFECTS
Succinic semialdehydedehydrogenase(1.2.1.24)4-Hydroxybutyric AciduriaMIM 271980
4-Aminobutyrateaminotransferase(2.6.1.19)GABA Transferase DeficiencyMIM 137150
UREA CYCLE DEFECTS(HYPERAMMONAEMIA)
Carbamoyl phosphatesynthase(6.3.4.16)CPS DeficiencyMIM 237300
Ornithine carbamoyltransferase(2.1.3.3)OCT DeficiencyMIM 300161
Argininosuccinate synthase(6.3.4.5)CitrullinaemiaMIM 215700
Argininosuccinate lyase(4.3.2.1)Argininosuccinate AciduriaMIM 207900
GLYCOGEN STORAGEDISEASES
MUSCLE GLYCOGENOSES
Muscle glycogenphosphorylase
(2.4.1.1)GSD Type V McArdle Disease
MIM 232600
LIVER GLYCOGENOSES
Amylo-1,6-glucosidase(3.2.1.33)
Glucanotransferase(2.4.1.25)
GSD Type III Cori DiseaseMIM 232400
Glucose-6-phosphatase(3.1.3.9)
GSD Type I Von GierkeDisease
MIM 232200
MONO- & DI-SACCHARIDES
Sucrase-isomaltase(3.2.1.10)
Sucrose-IsomaltoseMalabsorption
MIM 609845
Lactase(3.2.1.23) (3.2.1.108)Lactose Intolerance
MIM 223000
Galactokinase(2.7.1.6)
Galactose Kinase DeficiencyMIM 230200
Galactose-1-phosphateuridyl transferase
(2.7.7.12)Classical Galactosaemia
MIM 606999
PEROXISOMAL DEFECTS
Alanine-glyoxylateaminotransferase
(2.6.1.44)Hyperoxaluria Type 1
MIM 259900
LIPID METABOLISM
Hydroxymethylglutaryl CoAlyase
(4.1.3.4)Ketone Synthesis Defect
MIM 246450
Medium chain acyl CoAdehydrogenase
(1.3.99.3)MCAD Deficiency
MIM 607008
SPHINGOLIPIDOSES
Acid-ß-glucosidase(3.2.1.45)
Gaucher DiseaseMIM 230800
Hexosaminidase A(3.2.1.52)
Tay Sachs DiseaseMIM 272800
Sphingomyelinase(3.1.4.12)
Niemann-Pick DiseaseMIM 257200
RESPIRATORY CHAINDEFECTS
Cytochrome c oxidase(1.9.3.1)
Complex IVMIM 220110
PORPHYRIAS
Porphobilinogen deaminase(2.5.1.61)
Acute Intermittent PorphyriaMIM 176000
Uroporphyrinogendecarboxylase
(4.1.1.37)Porphyria Cutanea Tarda
MIM 176100
Protoporphyrinogen oxidase(1.3.3.4)
Variegate PorphyriaMIM 176200
sigma-aldrich.com/pathways