Amino acids levels and lipid peroxidation in maple syrup urine disease patients.
OBJECTIVE
In the present study we correlated the amino acids, branched-chain alpha-keto acids and alpha-hydroxy acids levels with the thiobarbituric acid-reactive species (TBARS) measurement, a lipid peroxidation parameter, in plasma from treated MSUD patients in order to examine whether these accumulated metabolites could be associated to the oxidative stress present in MSUD.


METHODS
TBARS, amino acids, branched-chain alpha-keto acids and alpha-hydroxy acids concentrations were measured in plasma samples from treated MSUD patients.


RESULTS
We verified that plasma TBARS was increased, whereas tryptophan and methionine concentrations were significantly reduced. Furthermore TBARS measurement was inversely correlated to methionine and tryptophan levels.


CONCLUSIONS
Considering that methionine and tryptophan have antioxidant activities, the data suggest that the imbalance of these amino acids may be involved with lipid peroxidation in MSUD.
Barschak
Alethéa G
AG
Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil. aletheagatto@gmail.com
Sitta
Angela
A
Deon
Marion
M
Busanello
Estela N B
EN
Coelho
Daniella M
DM
Cipriani
Franciele
F
Dutra-Filho
Carlos S
CS
Giugliani
Roberto
R
Wajner
Moacir
M
Vargas
Carmen R
CR
D000328:Adult; D000596:Amino Acids / Q000097:blood*; D000975:Antioxidants / Q000378:metabolism; D006801:Humans; D006880:Hydroxy Acids / Q000097:blood; D007532:Isoleucine / Q000097:blood; D007651:Keto Acids / Q000097:blood; D007930:Leucine / Q000097:blood; D015227:Lipid Peroxidation / Q000502:physiology*; D008375:Maple Syrup Urine Disease / Q000097:blood*; D008715:Methionine / Q000097:blood; D018384:Oxidative Stress; D017392:Thiobarbituric Acid Reactive Substances / Q000378:metabolism; D014364:Tryptophan / Q000097:blood; D014633:Valine / Q000097:blood
D000596:Amino Acids; D000975:Antioxidants; D006880:Hydroxy Acids; D007651:Keto Acids; D017392:Thiobarbituric Acid Reactive Substances; D007532:Isoleucine; D014364:Tryptophan; D008715:Methionine; D007930:Leucine; D014633:Valine