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Protein and lipid damage in maple syrup urine disease patients: l-carnitine effect.
Int J Dev Neurosci. 2013 Feb; 31(1):21-4.IJ

Abstract

Maple syrup urine disease (MSUD) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids (BCAA) leucine, isoleucine, valine and the corresponding branched-chain α-keto acids. This disorder is clinically characterized by ketoacidosis, seizures, coma, psychomotor delay and mental retardation whose pathophysiology is not completely understood. Recent studies have shown that oxidative stress may be involved in neuropathology of MSUD. l-Carnitine (l-Car) plays a central role in the cellular energy metabolism because it transports long-chain fatty acids for oxidation and ATP generation. In recent years many studies have demonstrated the antioxidant role of this compound. In this work, we investigated the effect of BCAA-restricted diet supplemented or not with l-Car on lipid peroxidation and in protein oxidation in MSUD patients. We found a significant increase of malondialdehyde and of carbonyl content in plasma of MSUD patients under BCAA-restricted diet compared to controls. Furthermore, patients under BCAA-restricted diet plus l-Car supplementation presented a marked reduction of malondialdehyde content in relation to controls, reducing the lipid peroxidation. In addition, free l-Car concentrations were negatively correlated with malondialdehyde levels. Our data show that l-Car may have an antioxidant effect, protecting against the lipid peroxidation and this could represent an additional therapeutic approach to the patients affected by MSUD.

Authors+Show Affiliations

Programa de Pós-Graduação em CB:Bioquímica e Departamento de Bioquímica, UFRGS, Rua Ramiro Barcelos, 2600, 90035-000 Porto Alegre, RS, Brazil. carolmescka@yahoo.com.brNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

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

Language

eng

PubMed ID

23137711

Citation

Mescka, Caroline Paula, et al. "Protein and Lipid Damage in Maple Syrup Urine Disease Patients: L-carnitine Effect." International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience, vol. 31, no. 1, 2013, pp. 21-4.
Mescka CP, Wayhs CA, Vanzin CS, et al. Protein and lipid damage in maple syrup urine disease patients: l-carnitine effect. Int J Dev Neurosci. 2013;31(1):21-4.
Mescka, C. P., Wayhs, C. A., Vanzin, C. S., Biancini, G. B., Guerreiro, G., Manfredini, V., Souza, C., Wajner, M., Dutra-Filho, C. S., & Vargas, C. R. (2013). Protein and lipid damage in maple syrup urine disease patients: l-carnitine effect. International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience, 31(1), 21-4. https://doi.org/10.1016/j.ijdevneu.2012.10.109
Mescka CP, et al. Protein and Lipid Damage in Maple Syrup Urine Disease Patients: L-carnitine Effect. Int J Dev Neurosci. 2013;31(1):21-4. PubMed PMID: 23137711.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Protein and lipid damage in maple syrup urine disease patients: l-carnitine effect. AU - Mescka,Caroline Paula, AU - Wayhs,Carlos Alberto Yasin, AU - Vanzin,Camila Simioni, AU - Biancini,Giovana Brondani, AU - Guerreiro,Gilian, AU - Manfredini,Vanusa, AU - Souza,Carolina, AU - Wajner,Moacir, AU - Dutra-Filho,Carlos Severo, AU - Vargas,Carmen Regla, Y1 - 2012/11/05/ PY - 2012/07/25/received PY - 2012/10/25/revised PY - 2012/10/25/accepted PY - 2012/11/10/entrez PY - 2012/11/10/pubmed PY - 2013/6/1/medline SP - 21 EP - 4 JF - International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience JO - Int J Dev Neurosci VL - 31 IS - 1 N2 - Maple syrup urine disease (MSUD) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids (BCAA) leucine, isoleucine, valine and the corresponding branched-chain α-keto acids. This disorder is clinically characterized by ketoacidosis, seizures, coma, psychomotor delay and mental retardation whose pathophysiology is not completely understood. Recent studies have shown that oxidative stress may be involved in neuropathology of MSUD. l-Carnitine (l-Car) plays a central role in the cellular energy metabolism because it transports long-chain fatty acids for oxidation and ATP generation. In recent years many studies have demonstrated the antioxidant role of this compound. In this work, we investigated the effect of BCAA-restricted diet supplemented or not with l-Car on lipid peroxidation and in protein oxidation in MSUD patients. We found a significant increase of malondialdehyde and of carbonyl content in plasma of MSUD patients under BCAA-restricted diet compared to controls. Furthermore, patients under BCAA-restricted diet plus l-Car supplementation presented a marked reduction of malondialdehyde content in relation to controls, reducing the lipid peroxidation. In addition, free l-Car concentrations were negatively correlated with malondialdehyde levels. Our data show that l-Car may have an antioxidant effect, protecting against the lipid peroxidation and this could represent an additional therapeutic approach to the patients affected by MSUD. SN - 1873-474X UR - https://www.unboundmedicine.com/medline/citation/23137711/Protein_and_lipid_damage_in_maple_syrup_urine_disease_patients:_l_carnitine_effect_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0736-5748(12)00582-5 DB - PRIME DP - Unbound Medicine ER -