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Propionyl-CoA is a key intermediate in the metabolism of odd-chain fatty acids and certain amino acids (methionine, valine, isoleucine, threonine). It is converted to methylmalonyl-CoA by propionyl-CoA carboxylase (PCC), then to succinyl-CoA by methylmalonyl CoA mutase, allowing entry into the citric acid cycle. Deficiency in PCC leads to propionic aciduria.
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