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Acknowledgements We thank Sarah Crittenden, Hannes Bulow, Alicia Melendez, and John Couchman and members of the Pocock Laboratory and for advice and comments on the manuscript

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The reason we use L-carnitine in VLCAD Deficiency is to maintain a reasonably normal ratio of Free Co-enzyme A to esterified Coenzyme A to facilitate mitochondrial metabolism during fat Page 2: mobilization due to fasting or unex