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When there are genetic variants in the PCCA or PCCB genes, propionyl CoA carboxylase is defective

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For instance, the mitochondrial shuttle-system allows the transport of fatty acids, as acylcarnitines, from the cytosol into the mitochondrial matrix where these nutrients are oxidized for ATP production

An overnight culture was used to inoculate 4 500 mL of LB medium containing 100 g ml 1 ampicillin (pETM20- msadh ) or 50 g ml 1 kanamycin (pETM11- msadh pETM11- mdh ), respectively

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