DES and acyl-CoA dehydrogenase deficiency: However, this patient showed an acyl CoA dehydrogenase deficiency-characteristic increase in the C8/C10-carnitine ratio [79] with a value of 0.98 (normal range, 0.60 to 0.00), a finding that was also present in our desmin knock-out mice (2.12 vs. 1.85, p = 0.049; Table 1).