“…Several reports have demonstrated that patients with hereditary organic acidaemias can develop a secondary carnitine deficiency, and that administration of camitine to these patients results in marked clinical improvement (Allen et al, 1982;Chalmers et al, 1983Chalmers et al, , 1984aRoe et al, 1983Roe et al, , 1984aDiDonato et al, 1984;Sugiyama et al, 1984). Evidence suggests that carnitine's efficacy is a result of acylcarnitine formation from the acyl-CoAs that accumulate secondary to the metabolic defect (Seccombe et al, 1982;Roe et al, 1983Roe et al, , 1984aDiDonato et al, 1984;Sugiyama et al, 1984;Chalmers et al, 1984a,b;Stumpfet al, 1985). This may remove acyl-CoAs that are metabolic inhibitors, such as propionyl-CoA (Glasgow & Chase, 1976;Gregersen, 1979Gregersen, , 1981Stumpfet al, 1980), as well as liberating CoA for utilization in other biochemical pathways not affected by the primary defect.…”