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Fatty Acid Oxidation Disorders

Background

Fatty acids are one of the body's fuels: oxidation is the process by which they are broken down to release energy. This process has many steps, each catalysed by a different enzyme. Fatty acid oxidation disorders result from deficiency of one of these enzymes:

  • Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency
  • Very-long-chain acyl-CoA dehydrogenase (VLCAD) deficiency
  • Short-chain acyl-CoA dehydrogenase (SCAD) deficiency
  • Multiple acyl-CoA dehydrogenase (MAD) deficiency (= Glutaric aciduria type II, GA II)
  • Long-chain hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency
  • Trifunctional protein deficiency
  • Carnitine palmitoyl-transferase I (CPT I) deficiency
  • Carnitine palmitoyl-transferase II (CPT II) deficiency
  • Carnitine acylcarnitine translocase deficiency
  • Primary (systemic) carnitine deficiency

What are the symptoms? View What are the symptoms?

Medical text written October 2000 by Dr A Morris. Last reviewed August 2005 by Dr A Morris, Consultant Paediatrician with Special Interest in Metabolic disease, Willink Biochemical Genetics Unit, Royal Manchester Children's Hospital, Manchester, UK.

 

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