Succinyl CoA: 3-Ketoacid CoA Transferase Deficiency

Metabolites

(R)-3-Hydroxybutanoate

Formula: C4H8O3 (104.0473418)

CAS ID: 625-72-9

Acetoacetate

Formula: C4H6O3 (102.0316926)

CAS ID: 541-50-4

Acetone

Formula: C3H6O (58.041862599999995)

CAS ID: 67-64-1

Acetyl-CoA

Formula: C23H38N7O17P3S (809.1257688000001)

CAS ID: 72-89-9

CO2

Formula: CO2 (43.98983)

CAS ID: 124-38-9

H2O

Formula: H2O (18.0105642)

CAS ID: 7732-18-5

NAD+

Formula: C21H28N7O14P2 (664.1169428000001)

CAS ID: 53-84-9

NADH

Formula: C21H29N7O14P2 (665.1247674)

CAS ID: 58-68-4



Enzyme

EC Number name full name note
1.1.1.146 11beta-hydroxysteroid dehydrogenase 11beta-hydroxysteroid:NADP+ 11-oxidoreductase
1.1.1.201 7beta-hydroxysteroid dehydrogenase (NADP+) 7beta-hydroxysteroid:NADP+ 7-oxidoreductase
2.3.3.10 hydroxymethylglutaryl-CoA synthase acetyl-CoA:acetoacetyl-CoA C-acetyltransferase (thioester-hydrolysing, carboxymethyl-forming)
2.8.3.5 3-oxoacid CoA-transferase succinyl-CoA:3-oxo-acid CoA-transferase
4.1.3.4 hydroxymethylglutaryl-CoA lyase (S)-3-hydroxy-3-methylglutaryl-CoA acetoacetate-lyase (acetyl-CoA-forming)


Proteins

Protein ID name full name