Step I MCAD deficiency

Started by ssj4vinh
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ssj4vinh

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From my understanding, medium and short chains (<14 carbon) do not need the carnitine shuttle to enter the mitochondria for beta oxidation. But a common finding in MCAD deficiency is increased 8-10 carbon fatty acyl carnitines in the blood. Why? Does it just back up the entire pathway and cause attachment of carnitine which never happened in the first place?
 
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