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Urinary biomarkers of oxidative damage in Maple syrup urine disease: the L-carnitine role.
Int J Dev Neurosci. 2015 May; 42:10-4.IJ

Abstract

Maple syrup urine disease (MSUD) is a disorder of branched-chain amino acids (BCAA). The defect in the branched-chain α-keto acid dehydrogenase complex activity leads to an accumulation of these compounds and their corresponding α-keto-acids and α-hydroxy-acids. Studies have shown that oxidative stress may be involved in neuropathology of MSUD. L-carnitine (L-car), which has demonstrated an important role as antioxidant by reducing and scavenging free radicals formation and by enhancing the activity of antioxidant enzymes, have been used in the treatment of some metabolic rare disorders. This study evaluated the oxidative stress parameters, di-tyrosine, isoprostanes and antioxidant capacity, in urine of MSUD patients under protein-restricted diet supplemented or not with L-car capsules at a dose of 50 mg kg(-1) day(-1). It was also determined urinary α-keto isocaproic acid levels as well as blood free L-car concentrations in blood. It was found a deficiency of carnitine in patients before the L-car supplementation. Significant increases of di-tyrosine and isoprostanes, as well as reduced antioxidant capacity, were observed before the treatment with L-car. The L-car supplementation induced beneficial effects on these parameters reducing the di-tyrosine and isoprostanes levels and increasing the antioxidant capacity. It was also showed a significant increase in urinary of α-ketoisocaproic acid after 2 months of L-car treatment, compared to control group. In conclusion, our results suggest that L-car may have beneficial effects in the treatment of MSUD by preventing oxidative damage to the cells and that urine can be used to monitorize oxidative damage in patients affected by this disease.

Authors+Show Affiliations

Faculdade de Farmácia, UFRGS, Av. Ipiranga 2752, 90610-000 Porto Alegre, RS, Brazil.Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, UFRGS, Rua Ramiro Barcelos, 2600, 90035-000 Porto Alegre, RS, Brazil. Electronic address: carolmescka@yahoo.com.br.Serviço de Genética Médica, HCPA, UFRGS, Rua Ramiro Barcelos, 2350, 90035-903 Porto Alegre, RS, Brazil.Programa de Pós-Graduação em Ciências Farmacêuticas, UFRGS, Av. Ipiranga, 2752, 90610-000 Porto Alegre, RS, Brazil.Serviço de Genética Médica, HCPA, UFRGS, Rua Ramiro Barcelos, 2350, 90035-903 Porto Alegre, RS, Brazil.Faculdade de Farmácia, UFRGS, Av. Ipiranga 2752, 90610-000 Porto Alegre, RS, Brazil.Faculdade de Farmácia, UFRGS, Av. Ipiranga 2752, 90610-000 Porto Alegre, RS, Brazil.Serviço de Genética Médica, HCPA, UFRGS, Rua Ramiro Barcelos, 2350, 90035-903 Porto Alegre, RS, Brazil.Serviço de Genética Médica, HCPA, UFRGS, Rua Ramiro Barcelos, 2350, 90035-903 Porto Alegre, RS, Brazil; Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, UFRGS, Rua Ramiro Barcelos, 2600, 90035-000 Porto Alegre, RS, Brazil.Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, UFRGS, Rua Ramiro Barcelos, 2600, 90035-000 Porto Alegre, RS, Brazil.Faculdade de Farmácia, UFRGS, Av. Ipiranga 2752, 90610-000 Porto Alegre, RS, Brazil; Serviço de Genética Médica, HCPA, UFRGS, Rua Ramiro Barcelos, 2350, 90035-903 Porto Alegre, RS, Brazil; Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, UFRGS, Rua Ramiro Barcelos, 2600, 90035-000 Porto Alegre, RS, Brazil; Programa de Pós-Graduação em Ciências Farmacêuticas, UFRGS, Av. Ipiranga, 2752, 90610-000 Porto Alegre, RS, Brazil.

Pub Type(s)

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

Language

eng

PubMed ID

25680940

Citation

Guerreiro, Gilian, et al. "Urinary Biomarkers of Oxidative Damage in Maple Syrup Urine Disease: the L-carnitine Role." International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience, vol. 42, 2015, pp. 10-4.
Guerreiro G, Mescka CP, Sitta A, et al. Urinary biomarkers of oxidative damage in Maple syrup urine disease: the L-carnitine role. Int J Dev Neurosci. 2015;42:10-4.
Guerreiro, G., Mescka, C. P., Sitta, A., Donida, B., Marchetti, D., Hammerschmidt, T., Faverzani, J., Coelho, D. d. e. . M., Wajner, M., Dutra-Filho, C. S., & Vargas, C. R. (2015). Urinary biomarkers of oxidative damage in Maple syrup urine disease: the L-carnitine role. International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience, 42, 10-4. https://doi.org/10.1016/j.ijdevneu.2015.02.003
Guerreiro G, et al. Urinary Biomarkers of Oxidative Damage in Maple Syrup Urine Disease: the L-carnitine Role. Int J Dev Neurosci. 2015;42:10-4. PubMed PMID: 25680940.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Urinary biomarkers of oxidative damage in Maple syrup urine disease: the L-carnitine role. AU - Guerreiro,Gilian, AU - Mescka,Caroline Paula, AU - Sitta,Angela, AU - Donida,Bruna, AU - Marchetti,Desirèe, AU - Hammerschmidt,Tatiane, AU - Faverzani,Jessica, AU - Coelho,Daniella de Moura, AU - Wajner,Moacir, AU - Dutra-Filho,Carlos Severo, AU - Vargas,Carmen Regla, Y1 - 2015/02/10/ PY - 2014/11/09/received PY - 2015/02/08/revised PY - 2015/02/08/accepted PY - 2015/2/15/entrez PY - 2015/2/15/pubmed PY - 2015/12/31/medline KW - Antioxidant KW - Maple syrup urine disease KW - Oxidative stress KW - Urine KW - l-Carnitine SP - 10 EP - 4 JF - International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience JO - Int J Dev Neurosci VL - 42 N2 - Maple syrup urine disease (MSUD) is a disorder of branched-chain amino acids (BCAA). The defect in the branched-chain α-keto acid dehydrogenase complex activity leads to an accumulation of these compounds and their corresponding α-keto-acids and α-hydroxy-acids. Studies have shown that oxidative stress may be involved in neuropathology of MSUD. L-carnitine (L-car), which has demonstrated an important role as antioxidant by reducing and scavenging free radicals formation and by enhancing the activity of antioxidant enzymes, have been used in the treatment of some metabolic rare disorders. This study evaluated the oxidative stress parameters, di-tyrosine, isoprostanes and antioxidant capacity, in urine of MSUD patients under protein-restricted diet supplemented or not with L-car capsules at a dose of 50 mg kg(-1) day(-1). It was also determined urinary α-keto isocaproic acid levels as well as blood free L-car concentrations in blood. It was found a deficiency of carnitine in patients before the L-car supplementation. Significant increases of di-tyrosine and isoprostanes, as well as reduced antioxidant capacity, were observed before the treatment with L-car. The L-car supplementation induced beneficial effects on these parameters reducing the di-tyrosine and isoprostanes levels and increasing the antioxidant capacity. It was also showed a significant increase in urinary of α-ketoisocaproic acid after 2 months of L-car treatment, compared to control group. In conclusion, our results suggest that L-car may have beneficial effects in the treatment of MSUD by preventing oxidative damage to the cells and that urine can be used to monitorize oxidative damage in patients affected by this disease. SN - 1873-474X UR - https://www.unboundmedicine.com/medline/citation/25680940/Urinary_biomarkers_of_oxidative_damage_in_Maple_syrup_urine_disease:_the_L_carnitine_role_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0736-5748(15)00011-8 DB - PRIME DP - Unbound Medicine ER -