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Fatty acids are activated for oxidation in the cytosol by the formation of their respective fatty acyl-CoA ester. The enzyme carnitine palmitoyltransferase 1A (CPT1A) is localized outside of the inner mitochondrial membrane and catalyzes the esterification of fatty acyl-CoA molecules to acyl-carnitine molecules.

Mutations in CPT1A result in inhibition of carnitine palmitoyltransferase 1A activity and consequently, inhibition of long chain fatty acid oxidation. However, oxidation of medium (8-10 Cs) and very long-chain (>22 Cs) fatty acid oxidation is unaffected.

Identify ALL of the following statements which correctly explain why only long chain fatty acid oxidation is affected by a mutation in CPT1A.

Select ALL that apply.


Medium chain fatty acids are oxidized in the cytosol and thus, are not dependent upon CPT1A.


Very long chain fatty acids are oxidized in the peroxisome and do not require carnitine for entry into the peroxisome.


Long chain fatty acyl-CoA molecules require CPT1A to form their respective acyl-carnitine molecules facilitating transfer across the inner mitochondrial membrane.


Medium chain fatty acids are able to diffuse across the mitochondrial membranes as fatty acyl-CoA molecules and, therefore, are not dependent upon CPT1A.


Only long chain fatty acids are metabolized in the mitochondria and thus dependent upon CPT1A activity for entry into the mitochondria.

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