2-Hydroxyglutric Aciduria (D and L Form)

Metabolites

2-Oxoglutarate

Formula: C5H6O5 (146.0215226)

CAS ID: 328-50-7

4-Aminobutanoate

Formula: C4H9NO2 (103.0633254)

CAS ID: 56-12-2

Acetyl-CoA

Formula: C23H38N7O17P3S (809.1257688000001)

CAS ID: 72-89-9

ADP

Formula: C10H15N5O10P2 (427.02941500000003)

CAS ID: 58-64-0

ADP-ribose

Formula: C15H23N5O14P2 (559.0716718)

CAS ID: 20762-30-5

Ammonia

Formula: NH3 (17.0265478)

CAS ID: 7664-41-7

AMP

Formula: C10H14N5O7P (347.06308240000004)

CAS ID: 61-19-8

ATP

Formula: C10H16N5O13P3 (506.9957476)

CAS ID: 56-65-5

Carbamoyl phosphate

Formula: CH4NO5P (140.9827104)

CAS ID: 590-55-6

CO2

Formula: CO2 (43.98983)

CAS ID: 124-38-9

D-Fructose 6-phosphate

Formula: C6H13O9P (260.0297178)

CAS ID: 643-13-0

Deamino-NAD+

Formula: C21H27N6O15P2 (665.1009592)

CAS ID: 6450-77-7

Glutathione

Formula: C10H17N3O6S (307.08380220000004)

CAS ID: 70-18-8

GMP

Formula: C10H14N5O8P (363.05799740000003)

CAS ID: 85-32-5

H+

Formula: H (1.0078246)

CAS ID: 12408-02-5

H2O

Formula: H2O (18.0105642)

CAS ID: 7732-18-5

L-Alanine

Formula: C3H7NO2 (89.0476762)

CAS ID: 56-41-7

L-Cysteine

Formula: C3H7NO2S (121.0197482)

CAS ID: 52-90-4

L-Glutamate

Formula: C5H9NO4 (147.0531554)

CAS ID: 56-86-0

L-Glutamine

Formula: C5H10N2O3 (146.069139)

CAS ID: 56-85-9

NAD+

Formula: C21H28N7O14P2 (664.1169428000001)

CAS ID: 53-84-9

NADH

Formula: C21H29N7O14P2 (665.1247674)

CAS ID: 58-68-4

NADP+

Formula: C21H29N7O17P3 (744.0832754)

CAS ID: 53-59-8

Orthophosphate

Formula: H3PO4 (97.9768968)

CAS ID: 7664-38-2

Oxaloacetate

Formula: C4H4O5 (132.00587339999998)

CAS ID: 328-42-7

Pyruvate

Formula: C3H4O3 (88.0160434)

CAS ID: 127-17-3

Succinate semialdehyde

Formula: C4H6O3 (102.0316926)

CAS ID: 692-29-5

Xanthosine 5'-phosphate

Formula: C10H13N4O9P (364.0420138)

CAS ID: 523-98-8

diphosphate

Formula: O7P2 (173.911931)

CAS ID: 14000-31-8

phosphate(3-)

Formula: O4P (94.953423)

CAS ID: 14265-44-2



Enzyme

EC Number name full name note
2.7.1.-
1.2.1.24 succinate-semialdehyde dehydrogenase (NAD+) succinate-semialdehyde:NAD+ oxidoreductase
1.2.1.88 L-glutamate gamma-semialdehyde dehydrogenase L-glutamate gamma-semialdehyde:NAD+ oxidoreductase
1.4.1.3 glutamate dehydrogenase [NAD(P)+] L-glutamate:NAD(P)+ oxidoreductase (deaminating)
1.8.1.7 glutathione-disulfide reductase glutathione:NADP+ oxidoreductase
2.1.3.2 aspartate carbamoyltransferase carbamoyl-phosphate:L-aspartate carbamoyltransferase
2.3.1.4 glucosamine-phosphate N-acetyltransferase acetyl-CoA:D-glucosamine-6-phosphate N-acetyltransferase
2.4.2.14 amidophosphoribosyltransferase 5-phospho-beta-D-ribosylamine:diphosphate phospho-alpha-D-ribosyltransferase (glutamate-amidating)
2.6.1.1 aspartate transaminase L-aspartate:2-oxoglutarate aminotransferase
2.6.1.16 glutamine-fructose-6-phosphate transaminase (isomerizing) L-glutamine:D-fructose-6-phosphate isomerase (deaminating)
2.6.1.19 4-aminobutyrate-2-oxoglutarate transaminase 4-aminobutanoate:2-oxoglutarate aminotransferase
2.6.1.2 alanine transaminase L-alanine:2-oxoglutarate aminotransferase
2.6.1.22 (S)-3-amino-2-methylpropionate transaminase (S)-3-amino-2-methylpropanoate:2-oxoglutarate aminotransferase
2.6.1.7 kynurenine-oxoglutarate transaminase L-kynurenine:2-oxoglutarate aminotransferase
2.7.1.59 N-acetylglucosamine kinase ATP:N-acetyl-D-glucosamine 6-phosphotransferase
2.7.1.60 N-acylmannosamine kinase ATP:N-acyl-D-mannosamine 6-phosphotransferase
3.5.1.2 glutaminase L-glutamine amidohydrolase
3.5.2.3 dihydroorotase (S)-dihydroorotate amidohydrolase
4.1.1.15 glutamate decarboxylase L-glutamate 1-carboxy-lyase (4-aminobutanoate-forming)
6.1.1.17 glutamate-tRNA ligase L-glutamate:tRNAGlu ligase (AMP-forming)
6.1.1.18 glutamine-tRNA ligase L-glutamine:tRNAGln ligase (AMP-forming)
6.1.1.24 glutamate-tRNAGln ligase L-glutamate:tRNAGlx ligase (AMP-forming)
6.3.2.2 glutamate-cysteine ligase L-glutamate:L-cysteine gamma-ligase (ADP-forming)
6.3.2.3 glutathione synthase gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
6.3.4.16 carbamoyl-phosphate synthase (ammonia) carbon-dioxide:ammonia ligase (ADP-forming, carbamate-phosphorylating)
6.3.5.2 GMP synthase (glutamine-hydrolysing) xanthosine-5'-phosphate:L-glutamine amido-ligase (AMP-forming)
6.3.5.5 carbamoyl-phosphate synthase (glutamine-hydrolysing) hydrogen-carbonate:L-glutamine amido-ligase (ADP-forming, carbamate-phosphorylating)


Proteins

Protein ID name full name