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Oxidative stress parameters in urine from patients with disorders of propionate metabolism: a beneficial effect of L:-carnitine supplementation.
Cell Mol Neurobiol. 2012 Jan; 32(1):77-82.CM

Abstract

Propionic (PA) and methylmalonic (MMA) acidurias are inherited disorders caused by deficiency of propionyl-CoA carboxylase and methylmalonyl-CoA mutase, respectively. Affected patients present acute metabolic crises in the neonatal period and long-term neurological deficits. Treatments of these diseases include a protein restricted diet and L: -carnitine supplementation. L: -Carnitine is widely used in the therapy of these diseases to prevent secondary L: -carnitine deficiency and promote detoxification, and several recent in vitro and in vivo studies have reported antioxidant and antiperoxidative effects of this compound. In this study, we evaluated the oxidative stress parameters, isoprostane and di-tyrosine levels, and the antioxidant capacity, in urine from patients with PA and MMA at the diagnosis, and during treatment with L: -carnitine and protein-restricted diet. We verified a significant increase of isoprostanes and di-tyrosine, as well as a significant reduction of the antioxidant capacity in urine from these patients at diagnosis, as compared to controls. Furthermore, treated patients presented a marked reduction of isoprostanes and di-tyrosine levels in relation to untreated patients. In addition, patients with higher levels of protein and lipid oxidative damage, determined by di-tyrosine and isoprostanes levels, also presented lower urinary concentrations of total and free L: -carnitine. In conclusion, the present results indicate that treatment with low protein diet and L: -carnitine significantly reduces urinary biomarkers of protein and lipid oxidative damage in patients with disorders of propionate metabolism and that L: -carnitine supplementation may be specially involved in this protection.

Authors+Show Affiliations

Programa de Pós-Graduação em Ciências Farmacêuticas, UFRGS, Ipiranga 2752, Porto Alegre, RS, 90610-000, Brazil. grazischmitt@terra.com.brNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Controlled Clinical Trial
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

21833551

Citation

Ribas, Graziela S., et al. "Oxidative Stress Parameters in Urine From Patients With Disorders of Propionate Metabolism: a Beneficial Effect of L:-carnitine Supplementation." Cellular and Molecular Neurobiology, vol. 32, no. 1, 2012, pp. 77-82.
Ribas GS, Biancini GB, Mescka C, et al. Oxidative stress parameters in urine from patients with disorders of propionate metabolism: a beneficial effect of L:-carnitine supplementation. Cell Mol Neurobiol. 2012;32(1):77-82.
Ribas, G. S., Biancini, G. B., Mescka, C., Wayhs, C. Y., Sitta, A., Wajner, M., & Vargas, C. R. (2012). Oxidative stress parameters in urine from patients with disorders of propionate metabolism: a beneficial effect of L:-carnitine supplementation. Cellular and Molecular Neurobiology, 32(1), 77-82. https://doi.org/10.1007/s10571-011-9736-8
Ribas GS, et al. Oxidative Stress Parameters in Urine From Patients With Disorders of Propionate Metabolism: a Beneficial Effect of L:-carnitine Supplementation. Cell Mol Neurobiol. 2012;32(1):77-82. PubMed PMID: 21833551.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Oxidative stress parameters in urine from patients with disorders of propionate metabolism: a beneficial effect of L:-carnitine supplementation. AU - Ribas,Graziela S, AU - Biancini,Giovana B, AU - Mescka,Caroline, AU - Wayhs,Carlos Y, AU - Sitta,Angela, AU - Wajner,Moacir, AU - Vargas,Carmen R, Y1 - 2011/07/22/ PY - 2011/02/05/received PY - 2011/06/30/accepted PY - 2011/8/12/entrez PY - 2011/8/13/pubmed PY - 2012/5/18/medline SP - 77 EP - 82 JF - Cellular and molecular neurobiology JO - Cell Mol Neurobiol VL - 32 IS - 1 N2 - Propionic (PA) and methylmalonic (MMA) acidurias are inherited disorders caused by deficiency of propionyl-CoA carboxylase and methylmalonyl-CoA mutase, respectively. Affected patients present acute metabolic crises in the neonatal period and long-term neurological deficits. Treatments of these diseases include a protein restricted diet and L: -carnitine supplementation. L: -Carnitine is widely used in the therapy of these diseases to prevent secondary L: -carnitine deficiency and promote detoxification, and several recent in vitro and in vivo studies have reported antioxidant and antiperoxidative effects of this compound. In this study, we evaluated the oxidative stress parameters, isoprostane and di-tyrosine levels, and the antioxidant capacity, in urine from patients with PA and MMA at the diagnosis, and during treatment with L: -carnitine and protein-restricted diet. We verified a significant increase of isoprostanes and di-tyrosine, as well as a significant reduction of the antioxidant capacity in urine from these patients at diagnosis, as compared to controls. Furthermore, treated patients presented a marked reduction of isoprostanes and di-tyrosine levels in relation to untreated patients. In addition, patients with higher levels of protein and lipid oxidative damage, determined by di-tyrosine and isoprostanes levels, also presented lower urinary concentrations of total and free L: -carnitine. In conclusion, the present results indicate that treatment with low protein diet and L: -carnitine significantly reduces urinary biomarkers of protein and lipid oxidative damage in patients with disorders of propionate metabolism and that L: -carnitine supplementation may be specially involved in this protection. SN - 1573-6830 UR - https://www.unboundmedicine.com/medline/citation/21833551/Oxidative_stress_parameters_in_urine_from_patients_with_disorders_of_propionate_metabolism:_a_beneficial_effect_of_L:_carnitine_supplementation_ L2 - https://doi.org/10.1007/s10571-011-9736-8 DB - PRIME DP - Unbound Medicine ER -