Pathway
Mitochondrial Fatty Acid Beta-Oxidation
Beta-oxidation begins once fatty acids have been imported into the mitochondrial matrix by carnitine acyltransferases. The beta-oxidation spiral of fatty acids metabolism involves the repetitive removal of two carbon units from the fatty acyl chain. There are four steps to this process: oxidation, hydration, a second oxidation, and finally thiolysis. The last step releases the two-carbon acetyl-CoA and a ready primed acyl-CoA that takes another turn down the spiral. In total each turn of the beta-oxidation spiral produces one NADH, one FADH2, and one acetyl-CoA.
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Further oxidation of acetyl-CoA via the tricarboxylic acid cycle generates additional FADH2 and NADH. All reduced cofactors are used by the mitochondrial electron transport chain to form ATP. The complete oxidation of a fatty acid molecule produces numerous ATP molecules. Palmitate, used as the model here, produces 129 ATPs.
Beta-oxidation pathways differ for saturated and unsaturated fatty acids. The beta-oxidation of saturated fatty acids requires four different enzymatic steps. Beta-oxidation produces and consumes intermediates with a trans configuration; unsaturated fatty acids that have bonds in the cis configuration require three separate enzymatic steps to prepare these molecules for the beta-oxidation pathway.
What the reference databases state
Statements
36 statements
36 statements · 1 reference database · Subject: Mitochondrial Fatty Acid Beta-Oxidation · Sources: Reactome
- ACAA2 participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- ACAD10 participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- ACAD11 participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- ACADL participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- ACADM participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- ACADS participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- ACADVL participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- ACBD6 participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- ACOT1 participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- ACOT11 participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- ACOT12 participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- ACOT13 participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
Show the remaining 24
- ACOT2 participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- ACOT7 participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- ACOT9 participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- ACSF2 participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- ACSM3 participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- ACSM6 participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- DBI participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- DECR1 participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- ECHS1 participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- ECI1 participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- HADH participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- HADHA participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- HADHB participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- MCAT participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- MCEE participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- MECR participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- MMAA participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- MMUT participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- NDUFAB1 participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- PCCA participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- PCCB participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- PCTP participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- THEM4 participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only
- THEM5 participates in Mitochondrial Fatty Acid Beta-OxidationProteinReactome R-HSA-77289Inferred only