Abstract
Recent publications have indicated the possibility of secondary carnitine deficiency and the need for supplemental L-carnitine in patients with disorders of organic acid metabolism (Roe and Bohun, 1982; Gregersen et al., 1982; Roe et al., 1983). L-Carnitine has a classical and important role in mediating the transport of long chain fatty acids into the mitochondrion for ?-oxidation and additional and possibly equally-important roles have been proposed for the modulation of intramitochondrial acyl-CoA/CoA ratios and the availability of free CoA., and in the detoxification and facilitated removal of excess and potentially toxic acyl groups from the mitochondrion and cell as acylcarnitine esters (Chalmers et al., 1983). Evidence for these roles has now been provided by our studies on patients with disorders of organic acid metabolism. In addition, the concept of a general approach to the treatment of patients with these disorders, that of L-carnitine therapy, has been developed from our observations.
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© 1984 SSIEM and MTP Press Limited, Queen Square, Lancaster
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Chalmers, R.A. et al. (1984). L-Carnitine Insufficiency in Disorders of Organic acid Metabolism: Response to L-Carnitine by Patients with Methylmalonic Aciduria and 3-Hydroxy-3-methylglutaric Aciduria. In: Addison, G.M., Chalmers, R.A., Divry, P., Harkness, R.A., Pollitt, R.J. (eds) Organic Acidurias. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-5612-4_29
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DOI: https://doi.org/10.1007/978-94-009-5612-4_29
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