Synthesis of Ketone Bodies

Sass JO. Inborn errors of ketogenesis and ketone body utilization. J Inherit Metab Dis. 2012 Jan;35(1):23–8. doi: 10.1007/s10545-011-9324-6. PMID: 21479626.

Metabolites

(R)-3-Hydroxybutanoate

Formula: C4H8O3 (104.0473418)

CAS ID: 625-72-9

(S)-3-Hydroxy-3-methylglutaryl-CoA

Formula: C27H44N7O20P3S (911.1574614)

CAS ID: 1553-55-5

Acetoacetate

Formula: C4H6O3 (102.0316926)

CAS ID: 541-50-4

H+

Formula: H (1.0078246)

CAS ID: 12408-02-5

Magnesium cation

Formula: Mg (23.98505)

CAS ID: 22537-22-0

acetoacetate

Formula: C4H5O3 (101.02386800000001)

CAS ID: 141-81-1

acetate

Formula: C2H3O2 (59.0133038)

CAS ID: 71-50-1

(3S)-3-hydroxy-3-methylglutaryl-CoA(5-)

Formula: C27H39N7O20P3S (906.1183384)

CAS ID:

adenosine 5'-monophosphate(2-)

Formula: C10H12N5O7P (345.0474332)

CAS ID:

ATP(4-)

Formula: C10H12N5O13P3 (502.9644492)

CAS ID:

NADH(2-)

Formula: C21H27N7O14P2 (663.1091182000001)

CAS ID:

NAD(1-)

Formula: C21H26N7O14P2 (662.1012936000001)

CAS ID: 53-84-9



Enzyme

EC Number name full name note
1.1.1.30 3-hydroxybutyrate dehydrogenase (R)-3-hydroxybutanoate:NAD+ oxidoreductase
2.3.1.9 acetyl-CoA C-acetyltransferase acetyl-CoA:acetyl-CoA C-acetyltransferase
2.3.3.10 hydroxymethylglutaryl-CoA synthase acetyl-CoA:acetoacetyl-CoA C-acetyltransferase (thioester-hydrolysing, carboxymethyl-forming)
4.1.3.4 hydroxymethylglutaryl-CoA lyase (S)-3-hydroxy-3-methylglutaryl-CoA acetoacetate-lyase (acetyl-CoA-forming)
6.2.1.1 acetate-CoA ligase acetate:CoA ligase (AMP-forming)
6.2.1.17 propionate-CoA ligase propanoate:CoA ligase (AMP-forming)


Proteins

Protein ID name full name