Cerebral organic acidurias, including diseases

Hoffmann GF, Böhles HJ, Burlina A, Duran M, Herwig J, Lehnert W, Leonard JV, Muntau A, Plecko-Starting FK, Superti-Furga A, Trefz FK, Christensen E. Early signs and course of disease of glutaryl-CoA dehydrogenase deficiency. J of Inher Metab Disea. 1995 Mar;18(2):173–6. doi: 10.1007/bf00711759.

Metabolites

(E)-Glutaconate

Formula: C5H6O4 (130.0266076)

CAS ID: 628-48-8

(S)-2-Hydroxyglutarate

Formula: C5H8O5 (148.0371718)

CAS ID: 13095-48-2

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

CoA

Formula: C21H36N7O16P3S (767.1152046000001)

CAS ID: 85-61-0

FAD

Formula: C27H33N9O15P2 (785.1571288000001)

CAS ID: 146-14-5

FADH2

Formula: C27H35N9O15P2 (787.1727780000001)

CAS ID: 1910-41-4

Glutarate

Formula: C5H8O4 (132.0422568)

CAS ID: 110-94-1

Glutaryl-CoA

Formula: C26H42N7O19P3S (881.1468972000001)

CAS ID: 3131-84-8

H+

Formula: H (1.0078246)

CAS ID: 12408-02-5

L-Aspartate

Formula: C4H7NO4 (133.0375062)

CAS ID: 56-84-8

L-Tryptophan

Formula: C11H12N2O2 (204.0898732)

CAS ID: 73-22-3

N-Acetyl-L-aspartate

Formula: C6H9NO5 (175.0480704)

CAS ID: 997-55-7

NAD+

Formula: C21H28N7O14P2 (664.1169428000001)

CAS ID: 53-84-9

NADH

Formula: C21H29N7O14P2 (665.1247674)

CAS ID: 58-68-4

NADPH

Formula: C21H30N7O17P3 (745.0911)

CAS ID: 2646-71-1

3-Hydroxyglutaric acid

Formula: C5H8O5 (148.0371718)

CAS ID: 638-18-6

2,6-Diaminohexanoic acid

Formula: C6H14N2O2 (146.1055224)

CAS ID: 70-54-2



Enzyme

EC Number name full name note
1.1.99.39 D-2-hydroxyglutarate dehydrogenase (R)-2-hydroxyglutarate:acceptor 2-oxidoreductase
1.3.8.6 glutaryl-CoA dehydrogenase (ETF) glutaryl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase (decarboxylating)


Proteins

Protein ID name full name