inborn errors of metabolism - erndim€¦ · 2h+ h+ oxaloacetate pyruvate succinyl-coa glutarate...

OXALOACETATE

PYRUVATE

SUCCINYL-CoA

GLUTARATE

CITRATE

MALATEOXO-2 - ASPARTATE

CH3COSCoA

ACETYL-CoA

TRANSAMINATION

5-Amino-levulinate

Glycine

1 αβ

c-sub-units

AD P+ Pi

3.6.1.34

H+-tra

nsporting ATP synthase

+ PDA iP

FUMARATE

NADH+H+CH2COO-C(OH)COO-CH2COO-

CH(OH)COO-CHCOO-

-OOCCOCH2CH2COO-

-OOCCH2CH2CO.SCoA

-OOCCOCH2COO-

CH3CH(OH)CH2CO.SCoA

-OOCCH=CHCOO-

Glyoxylate Cycle

-OOCCHO

GDPATP

HEME Protoporphyrinogen Coproporphyrinogen Uroporphyrinogen Porphobilinogen

5-Amino-levulinate

COO-CH2CH2

H2NCH2C=O

FADCyt.b

SUCCINATEII

CH3COCOO-

PYRUVATE

Cyt.c1

2e-2e-

2UQ UQ

UQH22UQH2

2UQ._ UQ.

Cyt.bL Cyt.bH

Heme a

Heme a3

2H+4H+

NADH+H+

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

ENDERGONIC REACTION

1.6. 5.3

2Fe -S (5 Clusters)

H+ H+H+ H+

PHOTO-SYSTEM

3.6.1.34

GlyceraldehydeRibulose-1,5-bis-P

H+ H+H+

H+H+H+H+

H+H+H+

H+H+H+Translocated protons

Ferredoxin

PCPQH2

2PQ_.Fe-S Cyt.f

Cyt bf

1e- 1e-

Cyclic Photophosphorylation

PHOTO-SYSTEM II

Cyt bc

Non-cyclic electron flow

2e-2e-

Protons from Water

2H+2H+

Fixation

NADPH+H+

ATPTHYLAKOID MEMBRANE

CHLOROPLAST OUTER MEMBRANE

THYLAKOID LUMEN

STROMA

tric current)

P680Chl.a

Pheophytin

Chl.A0

To Brain -

MIDINES

PURINES

CATECHOLAMINES

AROMATIC

hvVISION

PHOTOSYNTHESIS

PORPHYRINS

STEROIDS

ISOPRENOIDS

PHOSPHOLIPIDS

DEGRADATION

BIOSYNTHESIS

PENTOSES

HEXOSES

POLYSACCHARIDES

COCH CH(NH )COO2 3+

FOLICACID

C1POOL

VALINE

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

OHACNH

OHHO OPPU

NHCOCH

CH OH2

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

H H HO

HOCH2 C C C C CO

OH OHH OH

HOCH2 C C C CO 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

Glycerol

UDP-GalactoseUDP-Sugars

CH CH OH3 2ETHANOL

Dihydroxy-acetone-P

(Glycerone-P)

Glucosamine-6-P

Phospho-serine

Chain elongation Mitochondrial

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

Tryptamine 1-(o-Carboxy phenylamino)1-deoxyribulose-5-P

N-(5-P-Ribosyl)anthranilate

Anthranilate

Catechol

NHIndole-

acetaldehyde

CH CHO2

Indolepyruvate

C-CH(OH)CH(OH)CH O2 PCH

2-Aminomuconate-6-semialdehyde

CH CH NH2 2 2

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

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

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+

CH COCOO2

O-C-COOOH

OOC CH COCOO2

HOC-CH(OH)CH(OH)CH 2OPCH

OHNH2COO

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

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

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

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

H OHOCH2 C C C C CO

OHH OH

H OHOCH2 C C CO CO CO

NAD+Pi

ADPADP

Phytoene(C40)

Lycopene (C40)

ß-CAROTENE (C40)RhodopsinMetarhodopsin

Retinoatetrans-Retinal

11-cis-Retinoltrans-Retinol(Vitamin A)

Retinol esters

Ubiquinone(Coenzyme Q)

MenaquinonePlastoquinone

Phylloquinone(Vitamin K)

OpsinCHO

CH OH2

CH3CH O3CH O3 n

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)

CH CH NHCOCH2 2 3HO

CH CH NHCOCH2 2 3CH O3

+NAD( P)

NICOTINATE

N-Acetyl-serotonin

2.4.2.19

Dopamine Dopa

Dopaquinone

THYROXINEMELANIN

CHOHCH NHCH2 3

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

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

CH OH2

OHOCH3

CH(OH)COO

CH-COOCO

NRibose- PN+

CH COO2

CH O2 PNHCOCH NH2 2

OHFormyl

glycinamide-R P

Formylglycinamidine-R P

Allantoate Allantoin URATE

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

Carbamoylß-alanine

Dihydro-uracil Uracil

GTP GDP

XANTHOSINE-P(XMP)

ß-Ureidoisobutyrate

Dihydrothymine

Thymine d-UMP d-CMP d-CDP

Carbamoylaspartate

Dihydroorotate

Orotate Orotidine-P Uridine-P(UMP)

3-Amino-isobutyrate

CHCNH2

CHOCNH

CHOCN H

CH-CH3CO

CH2CH2

CHOCNH

HN C CH3

Methylmalonylsemialdehyde

OHCCHCOOCH3

CH-COOOCNH

HN CHCO

C-COOOCNH

H NCONHCH CH COO2 2 2

NH OOC-CH-CH COO2

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

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

4.2.1.59

4.2.1.58

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

CHOC N

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

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

CH CH NH2 2 2

Hydroxyphenylpyruvate

α-Tocopherol(Vitamin E)

LIGNIN

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

CH CH NH2 2 2

5.4.99.5

2.6.1.51.13.11.27

Indole

4.1.99.1

1.13.11.11

NH C C CH CH O2 P

H HCOO

6.3.5.3 6.3.2.6 4.3.2.2

GuanineDNA

6.3.4.44.3.2.2

HN CCO

3.5.3.4

2.4.2 1.

2.7.7.62.7.7.6

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

1.1.1.157

1.3.99.3

1.3.99.2

1.1.1.35Odd C Fatty acids

Succinylhomoserine

2-OXO ACID

Glutamyl-P

Glutamine

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

CH COCHCOSCoA3

CH3CH CH=CHCOSCoA3

CH3CH CH(OH)CHCOSCoA3

C(OH)CH(OH)COOCH CH3 2

CH = CCOSCoA2

CH3HOCH CHCOS-CoA-2

CH3CH CHCO-SCoA3

C (OH)CH(OH)COOCH3

CH CH CHCOSCoA3 2

CH C = CHCOSCoA3

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

(CH ) CHCHCH(OH)COO3 2

S-Adenosylmethylthiopropylamine

(Decarboxylated SAM)

PROLINE

Coenzyme A

CH C= CHCH3 2

CH3CH3

CH C-CH H O3 2 2C PPCH2

CH C= CHCH O2 2 PP

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

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

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

1.4.1.9

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

2.7.4.6

CYTIDINE-triphosphate

Cytosine

2.4.2.9

4.1.1.11

2.6.1.18

4.3.1.3

4.2.1.49

1.1.1.105

2.3.1.41

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

CH OH2

2.4.1.21

25.1.2.4

1.2.1.18

81.1.2.4

CHOC N

OHOHCHNHN

OCH2 C C

C CCNH2

RP(PP)

OCH2 C C

OHOHCO CH2 C

+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

CH OH2

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

OC N RP

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

4.2.1.16

Carnosine

CH CH NH2 2 2CNHN

4.1.1.22

4.3.1.3

Ubiquinone

Plastoquinone

D-Ribose-5-P

CH CH(NH )COO2 3HO+

HO OPPU

H OHHOCH2 C C CO

CO COO-

OCHOHCHOHAcNH

OCO -CH OH2

OCHOHCHOHAcNH

COOCH OH2

OHOHHOHO

CH O2 P

OHACNH

CH O2 P

OOHHOHO P

CH OH2

OHOH HOHO

CH OH2

OHHO OH

CH O2 PO

OHHO O

NHCOCH3

CH OH2CH OH2CH OH2

OHHO OH

CH O2 P

OHOPPU

CH OH2

CH OH2 O

CH OH2

CH2CH OP2

H H HOHC C C C

OH OHHCOO-HOCH2

OH OHOHC C C CHO

OH OHHC C C CHO

H OHHC C C CHO

OH HOHC C C CHO

C C C CHO

H OHOHC C C CHO

OH HOHC C C C

OHCOO-POCH2

H OHC C CO

POCH2 CH OH2

OH HC C CO

HOCH2 CH OH2

CH OH2POCH2

OH OHC C CO

CH OH2POCH2

H HC C CO

CH OH2POCH2

OH OHC C CO

HOCH2 CH OH2

H OHC C CO

HOCH2 CH OH2

H HC C CO

HOCH2 CH OH2

OH OHHC C C

HOCH2 CH OH2

H OHOHC C C

HOCH2 CH OH2

OH HOHC C C C

OHCH OH2HOCH2

OH OHC C CHO

HPOCH2

OH HOHC C C CO

HOCH2 CH O2 P

OH OHOHC C C

HOCH2 CH OH2

OH OHC C C

POCH2 CH OH2

OH OHC C C

HOCH O2 POCH2

OH OHC C

C CHOOH

POCH2POCH2

CH O2 P

CHOHCHOPOCH2HOCH2COCH2OP

OHO OP P

CH O2 PCHOHCOOPPOCH2

CHOHPOCH2 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 ) 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

O-CH O-PO2

CH CHOHCH OH2 2

R'-CO-OCH

O-CO-R

O-POO-

HOCH CH2 2NH3+POCH CH2 2NH3

+CPP- OCH CH2 2NH3

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+

CH CH 2 COO-(NH )3+

CH CH(NH )COO2 3+

NHOCCO

HC CHN

CHOC N

RP(P)HC N

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

CHCH(NH )COO3

++CNHN

CH CH(NH )CH OH2 3 2+

CH CH(NH )CH OP2 3 2CNHN

HC CH COCH OP2 2

+HSCH CH CH(NH )COO2 2 3

+SCH CH CH(NH )COO2 2 3

CH CH(NH )COO2 3

C-COOOCN

2.4.1.23

CH (CH ) CH=CHCH(OH)CHCH OH3 2 12 2

CH (CH ) CH(OH)CHCH OH3 2 14 2

CH (CH ) COCHCH OH3 2 14 2

GalactoseCH (CH ) CH=CHCH(OH)CHCH O-3 2 12 2

4.1.1.70

H NCONH2 2

OHCCH N(CH )2 3 3+

C-CH3CO

CHOCN 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

OCH CH3

COO-NHAC

HO OPPU

CH OH2

4.1.3.20

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

CHCHCH2

H C3H C3

CH2CH2COO- -

H C3NH

CH2CH2COO

CH2CH2COO -

CH 2CH 2

CH2CH2 CH2

COOCOO

CH2CH2COO

CH2CH2COO- -

OOC-CH3

CH2CH2COO

CH2CH2COO- -

CH C(OH)CH CH O3 2 2 PPCH COO2

OHCCOO

HOCH COO2

NH2H NCNHCH COO2 2

H NCN(CH )CH COO2 3 2

+ NH2HNCN(CH )CH COO3 2P-

+ HN CNH CO

OOCCHCH COO2

H NCNHCH CH CH CH2 2 2 2

N(NH )3+

CHCOOHCCH2HOCH

OOCCH(OH)CH CH(NH )COO2 3+

CHCOOCH2HOCH

H C2NH

CH2CH2CH2

OOCCH(OH)CH COCOO2

NCHCOO

CH2CH2

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

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

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+ +

HCCH-COO

CH2CH2

CH-COO OOCCN

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

OOCCH-CH CH CH CHCOO2 2 2NH3

(CH ) CHC(OH)CH3 2 2

COO (CH ) CHCH COCOO3 2 2

+(CH ) CHCH3 2 2 CH(NH )COO3

HOCH CHCOO2

CHCOCOOCH3

CH3CH COC(OH)CH3 3

+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

+CH(NH )COO3

CH COC(OH)CH CH3 2 3

COO CHCOCOOCH CH3 2

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

HC CH CHCOO2NHCOCH CH NH2 2 2

HC CH CH(NH )COO2 3

CHCH CH COO2 2NHN

OC C CH CHCOONHN

CH COO3

CH-COOOCNH2 CH2

H NCH CH COO2 2 2

H NCH2 2

CH3CHCOO H NCONHCH CHCOO2 2

CHRPHC N

RPH N2

OO P~ ~P O

O OP O

OP O O

H N2NH2

OOC-CH-CH COO2

HNCO CCH

OOCC CH

CH=CHCOO

CH COCOO2

CH COO2OCH COO2-OOC

OCH2 CH2

HOCH HCOH

NH2COOOCH

CH COCOO2NH

CHOCONH2

CH CH(NH )COO2 3+

COOCOO

HO OPPU

NHCOCH3

COOCH OH2

OCCH2 NHAC

HO OPPU

CH OH2

OHHO OHOH

OOCCH N(CH )2 3 2Dimethylglycine

1.1.99.1

1.2.1.8

COOHO HO COO

CH(O )PPOCH2 COO CH(OH)HOCH2 COOCOO

CH(O )PHOCH2 COO

CH =C(O )2 P COO

Ribose -O - P - O - P - O- Adenosine(P)+N

NRibose - O - P - O - P - O -Adenosine

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++

Prostaglandin PGE 2

HOCH2 C C CO C COO

OR'-CO-OCHCH O-CO-R2

CH O POCMP2O

CH CH N(CH )2 2 3+

OHOCHCH OPO2

CH OH2

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+

OHOCHCH OPO2

CH O-CO-R2

CH CH N(CH )2 2 3 3+

CH CH(NH )COO2 3+

CH CH(NH )CHO2 3

11-cis-Retinal

GDP-Mannuronate

Mannose-6-PDehydro-shikimate

Shikimate

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

P-Gluconolactone

1.1.1.44

CH O2 P

PHENYLALANINETYROSINE

TRYPTOPHAN

INOSINE-P(IMP)

THYMIDINE-P

HISTIDINE

URIDINE-triphosphate

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

OH OHC C C

POCH2 CH O2 P

5.4.2.1

4.2.1.11

Endoplasmic Reticulum

1.1.1.100

3.1.1.3

Phosphatidate

2.3.1.39

4.2.1.17

4.2.1.55

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

CH (CH ) CH=CHCH(OH)CHCH O3 2 12 2

NHAcylCH CH N(CH )2 2 3 3

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

SUCROSE

ADENOSINE-P(AMP)

TriacylglycerolFAT

OC-COOOH

CH CH 2 COO(NH )3+

GLYCOPROTEINSGANGLIOSIDES

MUCINS

4.1.2.-

2.6.1.-

GLUCOSE

O-Acyl-carnitine

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

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

+H NCNHCH CH CH CH2 2 2 2 COO(NH )3

OH HOHC C C CO

HOCH2 CH OH2

R-CH2COO

CH O-CO-R2

CH O-CO-R"2

R’-CO-OCH

CH CHNH2 3+

OR'-CO-OCH

CH O-CO-R2

CH O PO2

OOCCH CH CH2 2 COO(NH )3

+CH CH(OH)CH(NH )COO3 3

CH OH2CH OH2

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

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

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.

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

inborn errors of metabolism - erndim€¦ · 2h+ h+ oxaloacetate pyruvate succinyl-coa glutarate...

Documents

proyecto coa

product directory - bettshow.com · 94 pods & screens desks...

inborn errors of metabolism -...

aula 14 cinetica 4.ppt [modo de compatibilidade] · hmg-coa...

erndim / eugt meeting 5-6 october 2006, prague results of...

page 2 coa supplement no. 1 - home - coa

coa dof2009

introdução à bioquímica do rúmen - fcav.unesp.br · 4...

coa presentation5

coa presentation3

2013 proteochem catalog-rev1 · alternative name:...

coa carmelose

presentasi coa

metabolismo dos lipídios - files.cercomp.ufg.br ·...

sample coa application - federal aviation … · sample coa...

coa interupt

mucopolysaccharidoses - erndim€¦ ·...

metabolismo de lípidos - bioquimica kinesiologia unlam ·...

beta oxidación del acido linolenico. acil coa...

lecture (coa)