Rhabdomyolysis Caused by an Inherited Metabolic Disease: Very Long-chain Acyl-CoA Dehydrogenase Deficiency  Nicol C. Voermans, MD, Baziel G. van Engelen, MD, PhD, Leo A. Kluijtmans, PhD, Nike M. Stikkelbroeck, MD, PhD, Ad R. Hermus, MD, PhD  The American Journal of Medicine  Volume 119, Issue 2, Pages 176-179 (February 2006) DOI: 10.1016/j.amjmed.2005.07.064 Copyright © 2006 Elsevier Inc. Terms and Conditions

Figure 1 Mitochondrial β-oxidation. Activation of fatty-acids to acyl-CoA esters, followed by transport across the mitochondrial outer membrane. Transesterification to acylcarnitine by carnitine palmityl transferase I (CPT I). Transport across the inner membrane by carnitine acyl carnitine carrier. Re-esterification to acyl-CoA esters by CPT II. β-Oxidation of fatty acyl-CoA esters to generate acetyl-CoA and acyl-CoA shortened by two C-atoms. The first step is dehydrogenation, which is catalyzed by the acyl-CoA dehydrogenases, of which 3 different types have been identified, each named after their preferred substrate: very long-chain acyl-CoA dehydrogenase (VLCAD), medium chain acyl-CoA dehydrogenase (MCAD), or short-chain acyl-CoA dehydrogenase (SCAD). The next steps are hydration, oxidation, and thiolysis. This process is repeated until the fatty acid has been completely degraded to acetyl-CoA (C2), which then enters the citric acid cycle to generate adenosine triphosphate. Odd-numbered fatty acids are degraded to propionyl-CoA (C3), which also enters the citric acid cycle. The American Journal of Medicine 2006 119, 176-179DOI: (10.1016/j.amjmed.2005.07.064) Copyright © 2006 Elsevier Inc. Terms and Conditions