Inherited 3-methylglutaconic aciduria in two brothers--another defect of leucine metabolism.

@article{Durn1982Inherited3A,
  title={Inherited 3-methylglutaconic aciduria in two brothers--another defect of leucine metabolism.},
  author={M. X. Pe{\~n}a Dur{\'a}n and Frits A. Beemer and A S Tibosch and Lieneke Bruinvis and D. Ketting and Sybe K. Wadman},
  journal={The Journal of pediatrics},
  year={1982},
  volume={101 4},
  pages={
          551-4
        }
}
Two brothers, aged 7 and 5 years, who excreted large amounts of the leucine metabolites 3-methylglutaconic acid, 3-methylglutaric acid, and 3-hydroxyisovaleric acid, are described. The excretion of these metabolites could be enhanced by increasing the leucine intake. Restriction of the protein intake resulted in a marked reduction of the metabolite excretion. However, the excretion of the ultimate leucine metabolite, 3-hydroxy-3-methylglutaric acid, remained unchanged at a low level. The only… CONTINUE READING
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