Clinica chimica acta; international journal of clinical chemistry, 95(1), 11-16 (1979-07-02)
The urine of a child who presented with hyperammonemia was found to contain elevated levels of 3-hydroxy-3-methylglutaric acid, 3-methylglutaconic acid and 3-methylglutaric acid. An increased excretion of these organic acids has been reported previously in a child with 3-hydroxy-3-methylglutaryl-CoA lyase
The Journal of pediatrics, 101(4), 551-554 (1982-10-01)
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
What is the origin of 3-methylglutaconic acid?
R Walsh et al.
Journal of inherited metabolic disease, 22(3), 251-255 (1999-06-29)
European journal of pediatrics, 146(5), 484-488 (1987-09-01)
Persistent excretion of 3-methylglutaconic acid was found in a 6-month-old infant with multiple minor physical malformations and delayed development. During two episodes of intercurrent viral illness, the patient developed severe metabolic acidosis and excreted large amounts of lactate, 3-hydroxybutyrate and
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