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The shuttling of the beta-oxidation intermediates may occur at physical contacts between the two organelles 162 or potentially via as of yet uncharacterized bi-directional vesicular trafficking 163 , likely involving carnitine acetyl transferases 164

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Results develop gradually over 34 months and can last 2 years or more with a series of treatments

Given the significant changes in the liver after Cys restriction, we tested whether restoring Cse expression in a liver-specific manner can rescue the phenotype