DNA damage in an animal model of maple syrup urine disease

Molecular Genetics and Metabolism
Giselli ScainiEmilio L Streck

Abstract

Maple syrup urine disease is an inborn error of metabolism caused by a severe deficiency of the branched chain alpha-ketoacid dehydrogenase complex. Neurological dysfunction is a common finding in patients with maple syrup urine disease. However, the mechanisms underlying the neuropathology of brain damage in this disorder are poorly understood. In this study, we investigated whether acute or chronic administration of a branched chain amino acid pool (leucine, isoleucine and valine) causes transient DNA damage, as determined by the alkaline comet assay, in the brain and blood of rats during development and whether antioxidant treatment prevented the alterations induced by branched chain amino acids. Our results showed that the acute administration of branched chain amino acids increased the DNA damage frequency and damage index in the hippocampus. However, the chronic administration of branched chain amino acids increased the DNA damage frequency and damage index in both the hippocampus and the striatum, and the antioxidant treatment was able to prevent DNA damage in the hippocampus and striatum. The present study demonstrated that metabolite accumulation in MSUD induces DNA damage in the hippocampus and striatum and that it ma...Continue Reading

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Citations

Nov 14, 2013·Cellular and Molecular Neurobiology·Angela SittaCarmen R Vargas
Jun 15, 2014·Metabolism: Clinical and Experimental·Janne M StrandLars Eide
Nov 27, 2015·International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience·Luciana RosaEmilio L Streck
Apr 14, 2015·Mutation Research·Caroline Paula MesckaCarmen Regla Vargas
Nov 1, 2016·Molecular Neurobiology·Giselli ScainiEmilio L Streck

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