Mitochondrial Fatty Acid Beta-Oxidation

Rinaldo P, Matern D, Bennett MJ. Fatty acid oxidation disorders. Annu Rev Physiol. 2002;64():477–502. doi: 10.1146/annurev.physiol.64.082201.154705. PMID: 11826276.; Roe CR, Roe DS. Recent Developments in the Investigation of Inherited Metabolic Disorders Using Cultured Human Cells. Molecular Genetics and Metabolism. 1999 Oct;68(2):243–57. doi: 10.1006/mgme.1999.2911.; Stanley CA, Hale DE. Genetic disorders of mitochondrial fatty acid oxidation. Curr Opin Pediatr. 1994 Aug;6(4):476–81. doi: 10.1097/00008480-199408000-00021. PMID: 7951672.; Coates P, Tanaka K. Molecular basis of mitochondrial fatty acid oxidation defects. Journal of Lipid Research. 1992 Aug;33(8):1099–110. doi: 10.1016/s0022-2275(20)40762-x.

Metabolites

(S)-Hydroxyhexanoyl-CoA

Formula: C27H46N7O18P3S (881.1832806000001)

CAS ID: 79171-47-4

3-Oxotetradecanoyl-CoA

Formula: C35H60N7O18P3S (991.2928250000001)

CAS ID: 122364-86-7

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

Methylmalonyl-CoA

Formula: C25H40N7O19P3S (867.131248)

CAS ID: 1264-45-5

trans-3-Decenoyl-CoA

Formula: C31H52N7O17P3S (919.2353132)

CAS ID: 6410-54-4

2-trans-4-cis-decadienoyl-CoA

Formula: C31H50N7O17P3S (917.2196640000001)

CAS ID:

4Z-Decenoyl-CoA

Formula: C31H52N7O17P3S (919.2353132)

CAS ID: 69281-30-7

trans-Tetradec-2-enoyl-CoA

Formula: C35H60N7O17P3S (975.2979100000001)

CAS ID:

Adenosylcobalamin

Formula: C72H101CoN18O17P (1579.6661325999999)

CAS ID:

(S)-3-hydroxybutanoyl-CoA

Formula: C25H42N7O18P3S (853.1519822000001)

CAS ID:

(S)-3-hydroxypalmitoyl-CoA

Formula: C37H66N7O18P3S (1021.3397726)

CAS ID:

palmitoyl-CoA(4-)

Formula: C37H62N7O17P3S (1001.3135592)

CAS ID: 1763-10-6

butyryl-CoA(4-)

Formula: C25H38N7O17P3S (833.1257688000001)

CAS ID:

adenosine 5'-monophosphate(2-)

Formula: C10H12N5O7P (345.0474332)

CAS ID:

NADPH(4-)

Formula: C21H26N7O17P3 (741.0598016)

CAS ID: 53-57-6

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

ADP(3-)

Formula: C10H12N5O10P2 (424.0059412)

CAS ID:

NADP(3-)

Formula: C21H25N7O17P3 (740.051977)

CAS ID: 53-59-8



Enzyme

EC Number name full name note
1.1.1.35 3-hydroxyacyl-CoA dehydrogenase (S)-3-hydroxyacyl-CoA:NAD+ oxidoreductase
1.3.1.104 enoyl-[acyl-carrier-protein] reductase (NADPH) acyl-[acyl-carrier protein]:NADP+ oxidoreductase
2.3.1.16 acetyl-CoA C-acyltransferase acyl-CoA:acetyl-CoA C-acyltransferase
4.2.1.17 enoyl-CoA hydratase (3S)-3-hydroxyacyl-CoA hydro-lyase
6.4.1.3 propionyl-CoA carboxylase propanoyl-CoA:carbon-dioxide ligase (ADP-forming)


Proteins

Protein ID name full name