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With the mutations reported here, five different mutations (R19P, 133X, R169W, A301D, and W351R) have been detected in five different families in a relatively small geographical area consisting of Northern Italy and neighboring Switzerland

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Among these, FAs and their intracellular transport into the mitochondria is important for their use as fuel (Longo et al., 2016), which may become particularly important in the brain when glucose supply is reduced (Yao et al., 2011)