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Fat metabolism can also be regulated by malonyl-CoA-mediated inhibition of carnitine acyltransferase I. Such regulation serves to prevent de novo synthesized fatty acids from entering the mitochondria and being oxidized. Clinical Significance of Fatty Acids The majority of clinical problems related to fatty acid metabolism are associated with processes of oxidation. children These disorders fall into four main groups: 1. Deficiencies in children Carnitine: Deficiencies in carnitine lead to an inability to transport fatty children acids into the mitochondria for oxidation. This can occur in newborns and particularly in pre-term infants. Carnitine deficiencies also are found in patients undergoing hemodialysis or exhibiting organic aciduria. Carnitine deficiencies may manifest systemic symptomology or may be limited to only muscles. Symptoms can range from mild occasional muscle cramping to severe weakness or even death.
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