Reduction of lipid and protein damage in patients with disorders of propionate metabolism under treatment: a possible protective role of L-carnitine supplementation

作者：Ribas Graziela S^*; Manfredini Vanusa; de Mari Jurema F; Wayhs Carlos Y; Vanzin Camila S; Biancini Giovana B; Sitta Angela; Deon Marion; Wajner Moacir; Vargas Carmen R

来源：International Journal of Developmental Neuroscience, 2010, 28(2): 127-132.

DOI：10.1016/j.ijdevneu.2010.01.002

• Propionic acidemia
• Methylmalonic acidemia
• Oxidative damage
• L-Carnitine
• Free-radicals
• Antioxidants

Disorders of propionate metabolism are autosomal recessive diseases clinically characterized by acute metabolic crises in the neonatal period and long-term neurological deficits whose pathophysiology is not completely established. There are increasing evidences demonstrating antioxidant properties for L-carnitine, which is used in the treatment of propionic and methylmalonic acidemias to increase the excretion of organic acids accumulated in tissues and biological fluids of the affected patients. In this work we aimed to evaluate lipid (malondialdehyde content) and protein (carbonyl formation and sulfhydryl oxidation) oxidative damage in plasma from patients with propionic and methylmalonic acidemias at the moment of diagnosis and during treatment with L-carnitine. We also correlated the parameters of oxidative damage with plasma total, free and esterified L-carnitine levels. We found a significant increase of malondialdehyde and carbonyl groups, as well as a reduction of sulfhydryl groups in plasma of these patients at diagnosis compared to controls. Furthermore, patients under treatment presented a marked reduction of the content of protein carbonyl groups, similar to controls, and malondialdehyde content in relation to patients at diagnosis. In addition, plasma total and free L-carnitine concentrations were negatively correlated with malondialdehyde levels. Taken together, the present data indicate that treatment significantly reduces oxidative damage in patients affected by disorders of propionate metabolism and that L-carnitine supplementation may be involved in this protection.

• 出版日期2010-4