Diseases of metabolism

Metabolites

11-Deoxycortisol

Formula: C21H30O4 (346.214398)

CAS ID: 152-58-9

18-Hydroxycorticosterone

Formula: C21H30O5 (362.209313)

CAS ID: 561-65-9

6-Mercaptopurine

Formula: C5H4N4S (152.0156664)

CAS ID: 50-44-2

9-cis-Retinoic acid

Formula: C20H28O2 (300.2089188)

CAS ID: 5300-03-8

Adenine

Formula: C5H5N5 (135.054493)

CAS ID: 73-24-5

Androstenedione

Formula: C19H26O2 (286.1932696)

CAS ID: 63-05-8

beta-D-Galactose

Formula: C6H12O6 (180.0633852)

CAS ID: 7296-64-2

Bilirubin-glucuronoside

Formula: C39H44N4O12 (760.2955584)

CAS ID: 27071-67-6

Biotin

Formula: C10H16N2O3S (244.08815859999999)

CAS ID: 58-85-5

Calcidiol

Formula: C27H44O2 (400.3341124)

CAS ID: 19356-17-3

Calcitriol

Formula: C27H44O3 (416.3290274)

CAS ID: 32222-06-3

Calcium cation

Formula: Ca (39.962591)

CAS ID: 14127-61-8

Corticosterone

Formula: C21H30O4 (346.214398)

CAS ID: 50-22-6

Estradiol-17beta

Formula: C18H24O2 (272.17762039999997)

CAS ID: 50-28-2

Fe2+

Formula: Fe (55.934939)

CAS ID: 15438-31-0

Galactose

Formula: C6H12O6 (180.0633852)

CAS ID: 26566-61-0

Glutathione

Formula: C10H17N3O6S (307.08380220000004)

CAS ID: 70-18-8

H+

Formula: H (1.0078246)

CAS ID: 12408-02-5

H2O

Formula: H2O (18.0105642)

CAS ID: 7732-18-5

HCO3-

Formula: HCO3 (60.9925696)

CAS ID: 71-52-3

Isomaltose

Formula: C12H22O11 (342.11620619999997)

CAS ID: 499-40-1

Magnesium cation

Formula: Mg (23.98505)

CAS ID: 22537-22-0

Manganese(2+)

Formula: Mn (54.938046)

CAS ID: 7439-96-5

Methylmalonyl-CoA

Formula: C25H40N7O19P3S (867.131248)

CAS ID: 1264-45-5

NADPH

Formula: C21H30N7O17P3 (745.0911)

CAS ID: 2646-71-1

Oxygen

Formula: O2 (31.98983)

CAS ID: 7782-44-7

Potassium cation

Formula: K (38.963708)

CAS ID: 24203-36-9

Pregnenolone

Formula: C21H32O2 (316.24021719999996)

CAS ID: 145-13-1

Progesterone

Formula: C21H30O2 (314.224568)

CAS ID: 57-83-0

Retinoate

Formula: C20H28O2 (300.2089188)

CAS ID: 302-79-4

Sodium cation

Formula: Na (22.98977)

CAS ID: 17341-25-2

Sucrose

Formula: C12H22O11 (342.11620619999997)

CAS ID: 57-50-1

Sulfate

Formula: H2SO4 (97.9673812)

CAS ID: 7664-93-9

Trimethylamine

Formula: C3H9N (59.0734954)

CAS ID: 75-50-3

Vitamin D3

Formula: C27H44O (384.3391974)

CAS ID: 67-97-0

Zinc cation

Formula: Zn (63.929145)

CAS ID: 23713-49-7

Hebevinoside I

Formula: C44H72O13 (808.4972662)

CAS ID: 87396-30-3

D-fructose 1-phosphate

Formula: C6H13O9P (260.0297178)

CAS ID: 15978-08-2

L-argininium(1+)

Formula: C6H15N4O2 (175.119495)

CAS ID: 17806-42-7

D-glucopyranuronate

Formula: C6H9O7 (193.0348264)

CAS ID: 148-00-5

2-oxoglutarate(2-)

Formula: C5H4O5 (144.00587339999998)

CAS ID: 64-15-3

NADPH(4-)

Formula: C21H26N7O17P3 (741.0598016)

CAS ID: 53-57-6

ATP(4-)

Formula: C10H12N5O13P3 (502.9644492)

CAS ID:

NAD(1-)

Formula: C21H26N7O14P2 (662.1012936000001)

CAS ID: 53-84-9

NADP(3-)

Formula: C21H25N7O17P3 (740.051977)

CAS ID: 53-59-8



Enzyme

EC Number name full name note
1.14.13.-
1.1.1.10 L-xylulose reductase xylitol:NADP+ 4-oxidoreductase (L-xylulose-forming)
1.1.1.42 isocitrate dehydrogenase (NADP+) isocitrate:NADP+ oxidoreductase (decarboxylating)
1.14.14.24 vitamin D 25-hydroxylase calciol,NADPH-hemoprotein reductase:oxygen oxidoreductase (25-hydroxylating)
1.14.16.1 phenylalanine 4-monooxygenase L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase (4-hydroxylating)
1.16.1.6 cyanocobalamin reductase cob(II)alamin, hydrogen cyanide:NADP+ oxidoreductase
1.16.1.8 [methionine synthase] reductase [methionine synthase]-methylcob(III)alamin,S-adenosyl-L-homocysteine:NADP+ oxidoreductase
1.18.1.6 adrenodoxin-NADP+ reductase adrenodoxin:NADP+ oxidoreductase
1.2.4.4 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) 3-methyl-2-oxobutanoate:[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]-lipoyllysine 2-oxidoreductase (decarboxylating, acceptor-2-methylpropanoylating)
1.4.3.21 primary-amine oxidase primary-amine:oxygen oxidoreductase (deaminating)
1.4.3.4 monoamine oxidase amine:oxygen oxidoreductase (deaminating)
1.8.1.4 dihydrolipoyl dehydrogenase protein-N6-(dihydrolipoyl)lysine:NAD+ oxidoreductase
2.1.1.13 methionine synthase 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
2.2.1.2 transaldolase sedoheptulose-7-phosphate:D-glyceraldehyde-3-phosphate glyceronetransferase
2.3.1.1 amino-acid N-acetyltransferase acetyl-CoA:L-glutamate N-acetyltransferase
2.3.1.168 dihydrolipoyllysine-residue (2-methylpropanoyl)transferase 2-methylpropanoyl-CoA:enzyme-N6-(dihydrolipoyl)lysine S-(2-methylpropanoyl)transferase
2.4.1.109 dolichyl-phosphate-mannose-protein mannosyltransferase dolichyl-phosphate-D-mannose:protein O-D-mannosyltransferase
2.4.1.11 glycogen(starch) synthase UDP-glucose:glycogen 4-alpha-D-glucosyltransferase
2.4.1.122 N-acetylgalactosaminide beta-1,3-galactosyltransferase UDP-alpha-D-galactose:N-acetyl-alpha-D-galactosaminyl-R beta-1,3-galactosyltransferase (configuration-inverting)
2.4.1.135 galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase UDP-alpha-D-glucuronate:[protein]-3-O-(beta-D-galactosyl-(1->3)-beta-D-galactosyl-(1->4)-beta-D-xylosyl)-L-serine D-glucuronosyltransferase (configuration-inverting)
2.4.1.141 N-acetylglucosaminyldiphosphodolichol N-acetylglucosaminyltransferase UDP-N-acetyl-D-glucosamine:N-acetyl-D-glucosaminyl-diphosphodolichol N-acetyl-D-glucosaminyltransferase
2.4.1.17 glucuronosyltransferase UDP-glucuronate beta-D-glucuronosyltransferase (acceptor-unspecific)
2.4.1.186 glycogenin glucosyltransferase UDP-alpha-D-glucose:glycogenin alpha-D-glucosyltransferase
2.4.1.22 lactose synthase UDP-galactose:D-glucose 4-beta-D-galactosyltransferase
2.4.1.222 O-fucosylpeptide 3-beta-N-acetylglucosaminyltransferase UDP-N-acetyl-alpha-D-glucosamine:[protein with EGF-like domain]-3-O-(alpha-L-fucosyl)-(L-serine/L-threonine) 3-beta-N-acetyl-D-glucosaminyltransferase (configuration-inverting)
2.4.1.224 glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase UDP-N-acetyl-D-glucosamine:beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase
2.4.1.225 N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase UDP-alpha-D-glucuronate:N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan 4-beta-glucuronosyltransferase
2.4.1.275 neolactotriaosylceramide beta-1,4-galactosyltransferase UDP-alpha-D-galactose:N-acetyl-beta-D-glucosaminyl-(1->3)-beta-D-galactosyl-(1beta4)-beta-D-glucosyl-(1<->1)-ceramide 4-beta-D-galactosyltransferase
2.4.1.38 beta-N-acetylglucosaminylglycopeptide beta-1,4-galactosyltransferase UDP-galactose:N-acetyl-beta-D-glucosaminylglycopeptide 4-beta-galactosyltransferase
2.4.1.83 dolichyl-phosphate beta-D-mannosyltransferase GDP-mannose:dolichyl-phosphate beta-D-mannosyltransferase
2.4.1.90 N-acetyllactosamine synthase UDP-galactose:N-acetyl-D-glucosamine 4-beta-D-galactosyltransferase
2.4.2.1 purine-nucleoside phosphorylase purine-nucleoside:phosphate ribosyltransferase
2.4.2.7 adenine phosphoribosyltransferase AMP:diphosphate phospho-D-ribosyltransferase
2.4.2.8 hypoxanthine phosphoribosyltransferase IMP:diphosphate phospho-D-ribosyltransferase
2.5.1.151 alkylcobalamin dealkylase methylcobalamin:glutathione S-methyltransferase
2.5.1.87 ditrans,polycis-polyprenyl diphosphate synthase [(2E,6E)-farnesyl diphosphate specific] (2E,6E)-farnesyl-diphosphate:isopentenyl-diphosphate cistransferase (adding 10–55 isopentenyl units)
2.7.1.3 ketohexokinase ATP:D-fructose 1-phosphotransferase
2.7.11.1 non-specific serine/threonine protein kinase ATP:protein phosphotransferase (non-specific)
2.7.11.4 [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] kinase ATP:[3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] phosphotransferase
3.1.3.16 protein-serine/threonine phosphatase protein-serine/threonine-phosphate phosphohydrolase
3.1.3.48 protein-tyrosine-phosphatase protein-tyrosine-phosphate phosphohydrolase
3.1.3.52 [3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)]-phosphatase [3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)]-phosphate phosphohydrolase
3.2.1.108 lactase lactose galactohydrolase (configuration-retaining)
3.2.1.23 beta-galactosidase beta-D-galactoside galactohydrolase
3.2.1.52 beta-N-acetylhexosaminidase beta-N-acetyl-D-hexosaminide N-acetylhexosaminohydrolase
3.2.1.62 glycosylceramidase beta-D-glucosyl-N-acylsphingosine glycohydrolase (configuration-retaining)
3.4.16.5 carboxypeptidase C
4.1.2.13 fructose-bisphosphate aldolase D-fructose-1,6-bisphosphate D-glyceraldehyde-3-phosphate-lyase (glycerone-phosphate-forming)
5.1.3.2 UDP-glucose 4-epimerase UDP-glucose 4-epimerase
5.1.3.7 UDP-N-acetylglucosamine 4-epimerase UDP-N-acetyl-D-glucosamine 4-epimerase
5.3.1.6 ribose-5-phosphate isomerase D-ribose-5-phosphate aldose-ketose-isomerase
5.4.99.2 methylmalonyl-CoA mutase (R)-methylmalonyl-CoA CoA-carbonylmutase
6.4.1.1 pyruvate carboxylase pyruvate:carbon-dioxide ligase (ADP-forming)
6.4.1.2 acetyl-CoA carboxylase acetyl-CoA:hydrogencarbonate ligase (ADP-forming)
6.4.1.3 propionyl-CoA carboxylase propanoyl-CoA:carbon-dioxide ligase (ADP-forming)
6.4.1.4 methylcrotonoyl-CoA carboxylase 3-methylcrotonoyl-CoA:carbon-dioxide ligase (ADP-forming)
7.6.2.8 ABC-type vitamin B12 transporter ATP phosphohydrolase (ABC-type, vitamin B12-importing)


Proteins

Protein ID name full name