It is formed as a product of beta-oxidation of odd-chain fatty acids.

Unsaturated and odd-chain fatty acids require additional enzymatic steps for degradation.

These compounds are normally produced during bodily metabolism of amino acids and odd-chain fatty acids, but accumulate in patients with certain metabolic conditions.

This reaction, which requires vitamin B as a cofactor, is important in the catabolism of some branched-chain amino acids as well as odd-chain fatty acids.

Propionyl CoA is the end product of metabolism of odd-chain fatty acids, and is also a metabolite of most methyl-branched fatty acids.

Deoxyadenosylcobalamin is required in the metabolism of odd-chain fatty acids in mitochondria and in the synthesis of fatty acids in nervous tissue (myelin sheath formation).

It is known that odd-chain fatty acids can be oxidized to yield propionyl-CoA, a precursor for succinyl-CoA, which can be converted to pyruvate and enter into gluconeogenesis.

The percentages of odd-chain fatty acids (C15 and C17), unsaturated fatty acids (especially the trans form), and isooctadecanoic acid were significantly greater in the mutant strain than the parent strain.

The essential amino acids isoleucine, valine, threonine, and methionine, as well as odd-chain fatty acids, are simply converted to propionyl CoA, before the process stops, leading to a buildup of propionyl CoA.

The substrate of methylmalonyl-CoA mutase, methylmalonyl-CoA, is primarily derived from propionyl-CoA, a substance formed from the catabolism and digestion of isoleucine, valine, threonine, methionine, thymine, cholesterol, or odd-chain fatty acids.