A chemically-induced acute model of maple syrup urine disease in rats for neurochemical studies

Journal of Neuroscience Methods
Raquel BridiCarlos Severo Dutra-Filho

Abstract

We report a chemically-induced model of maple syrup urine disease (MSUD) in 10- and 30-day-old rats produced by subcutaneous administration of a branched-chain amino acids (BCAA) pool along with the analyses of plasma and brain amino acid levels by HPLC at 0-120 min after administration. We observed an increase of plasma leucine (Leu), isoleucine (Ile) and valine (Val) concentrations in both 10- and 30-day-old rats. These increases were accompanied by a concomitant reduction of plasma concentrations of methionine (Met), phenylalanine (Phe), tyrosine (Tyr), histidine (His), alanine (Ala), lysine (Lys), and ornithine (Orn) in 10-day-old rats and of Met, Phe, Tyr, tryptophan (Trp), and Orn in 30-day-old rats. These results are similar to those observed in MSUD patients during crises, when plasma levels of large neutral amino acids (LNAA) are also reduced when BCAA concentrations are increased. In the brain, increased concentrations of Leu, Ile and Val were achieved in 10-day-old rats at all times after injection. In contrast, no differences in cerebral concentrations of BCAA were observed in 30-day-old rats. In conclusion, the present MSUD model, using 10- rather than 30-day-old rats, has a similar amino acid profile to that of MS...Continue Reading

References

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Apr 25, 2003·International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience·Carmen PillaClóvis Milton Duval Wannmacher
Apr 29, 2003·Neurochemical Research·Carmen PillaClóvis Milton Duval Wannmacher
Aug 21, 2003·International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience·Raquel BridiCarlos Severo Dutra-Filho
May 7, 2005·Free Radical Research·Raquel BridiCarlos Severo Dutra-Filho
Jun 3, 2005·The Journal of Nutrition·John D Fernstrom

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Citations

Nov 14, 2013·Cellular and Molecular Neurobiology·Angela SittaCarmen R Vargas
Jun 29, 2010·Cellular and Molecular Neurobiology·Viviane GlaserAlexandra Latini
Feb 14, 2012·Molecular Neurobiology·Giselli ScainiEmilio L Streck
Nov 27, 2015·International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience·Luciana RosaEmilio L Streck
May 9, 2012·Molecular Genetics and Metabolism·Giselli ScainiEmilio L Streck
Mar 22, 2012·Behavioural Brain Research·Giselli ScainiEmilio L Streck
Feb 15, 2015·International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience·Gilian GuerreiroCarmen Regla Vargas
Nov 1, 2016·Molecular Neurobiology·Caroline Paula MesckaCarlos Severo Dutra-Filho
Nov 14, 2015·Metabolic Brain Disease·Rodrigo Binkowski de AndradeClovis Milton Duval Wannmacher
Mar 8, 2011·Metabolic Brain Disease·Caroline MesckaCarlos S Dutra-Filho

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