- Deficient beta-oxidation of fatty acids can be produced by carnitine deficiency or genetic defects in the translocase or other enzymes involved in the transfer of long-chain fatty acids into the mitochondria.  This causes cardiomyopathy.  In addition, it causes hypoketonemic hypoglycemia with coma, a serious and often fatal condition triggered by fasting, in which glucose stores are used up because of the lack of fatty acid oxidation to provide energy.  Ketone bodies are not formed in normal amounts because of the lack of adequate CoA in the liver.

Reference:
1. Ganong's Review of Medical Physiology: 23rd Edition.  Kim E. Barrett et al.  McGraw-Hill Publishing. 2010.