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Using isolated hepatocytes, our study establishes an authentic human model for MMA that recapitulates the known molecular features of the disorder, such as depletion of TCA cycle intermediates and accumulation of metabolites proximal to the impaired enzymatic activity

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We excluded Lenzi 2003 as it had a cross-over design (Lenzi et al

L-carnitine is formed in the liver and kidneys from the amino acids lysine and methionine

44 Furthermore, alterations in brain omega 3/6 cardiolipin profiles found in the PPA rodent model could change inner mitochondrial membrane fluidity, and, thus, could potentially affect mitochondrial ETC complex interactions