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This complex is then escorted across the inner membrane by a specific transporter protein

Mykkanen L, Kuusisto J, Haffner SM, Laakso M, Austin MA

It is caused by a pure or compound heterozygous genetic variant in the MLYCD gene located on chromosome 16q24 [22]
68 Some of these effects contribute to the toxicity we see with typical overdoses: CNS depression, lethargy, coma, and seizures

This low insulin state produces an increase in free fatty acids that are taken up by the mitochondria for the consequent increase in ketone body synthesis
