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These include subgroups with hyperactive mitochondrial respiration, partial disfunction, or classical mitochondrial disease patterns, each potentially associated with different clinical features such as neurodevelopmental regression or immune malfunction (Frye et al., 2024)

In this metabolic pathway, the cofactor carnitine is crucial since it serves as a shuttle-molecule to allow fatty acid acyl moieties entering the mitochondrial matrix where these molecules are oxidized via the -oxidation pathway

Am.J Kidney Dis 2008;52(5):962-971