hypoketotic hypoglycemia

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Aconitase

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Why do you get hypoglycemia with carnitine deficiency? i understand the hypoketotic part but not so much the hypoglycemic part. I can't find a good explanation anywhere.

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The carbons of glucose can be made into the carbons of fatty acids but the opposite isn't true. However, there is a relation between beta-oxidation and gluconeogenesis because the ENERGY (not the carbons) from beta-oxidation is what will be used to fuel gluconeogenesis and if beta-oxidation is messed up, can't get energy to drive gluconeogenesis hence hypoglycemia.
 
Carnitine deficiency --> decreased production of acetyl-CoA. Since acetyl-CoA is a co-factor for pyruvate carboxylase (the first step in gluconeogenesis) --> decreased glucose production.

Knowing acetyl-CoA is a co-factor for that reaction is mega-high-yield.
 
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