Glutaric Aciduria Type I

Metabolites

2-Oxoadipate

Formula: C6H8O5 (160.0371718)

CAS ID: 3184-35-8

2-Oxoglutarate

Formula: C5H6O5 (146.0215226)

CAS ID: 328-50-7

Acetyl-CoA

Formula: C23H38N7O17P3S (809.1257688000001)

CAS ID: 72-89-9

CO2

Formula: CO2 (43.98983)

CAS ID: 124-38-9

H+

Formula: H (1.0078246)

CAS ID: 12408-02-5

H2O

Formula: H2O (18.0105642)

CAS ID: 7732-18-5

Hydrogen peroxide

Formula: H2O2 (34.0054792)

CAS ID: 7722-84-1

L-Glutamate

Formula: C5H9NO4 (147.0531554)

CAS ID: 56-86-0

L-Lysine

Formula: C6H14N2O2 (146.1055224)

CAS ID: 56-87-1

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

Oxygen

Formula: O2 (31.98983)

CAS ID: 7782-44-7

L-2-Amino-6-oxohexanoic acid

Formula: C6H11NO3 (145.0738896)

CAS ID: 1962-83-0



Enzyme

EC Number name full name note
1.1.1.35 3-hydroxyacyl-CoA dehydrogenase (S)-3-hydroxyacyl-CoA:NAD+ oxidoreductase
1.2.1.24 succinate-semialdehyde dehydrogenase (NAD+) succinate-semialdehyde:NAD+ oxidoreductase
1.3.8.6 glutaryl-CoA dehydrogenase (ETF) glutaryl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase (decarboxylating)
1.8.1.7 glutathione-disulfide reductase glutathione:NADP+ oxidoreductase
4.2.1.17 enoyl-CoA hydratase (3S)-3-hydroxyacyl-CoA hydro-lyase
5.3.3.8 DELTA3-DELTA2-enoyl-CoA isomerase (3Z/3E)-alk-3-enoyl-CoA (2E)-isomerase


Proteins

Protein ID name full name