Demonstration of N-dicarboxyl-mono-glycines in dicarboxylic acidurias by mass fragmentography.
Urine samples from 18 individuals with various types of dicarboxylic acidurias have been investigated by mass fragmentography for N-dicarboxyl-mono-glycines (dicarboxylglycines). One patient with methylmalonic acidemia excreted 14-20 microgram methylmalonylglycine/mg creatinine, three patients with glutaric aciduria excreted 20-60 microgram glutarylglycine/creatinine, and one patient with C6-C10-dicarboxylic aciduria excreted 120-365 microgram succinylglycine/mg creatinine. Excretion of C6-C10-dicarboxylic acids in patients with ketosis and glycogenosis and in neonates were not accompanied by excretion of C8-C10-dicarboxylglycines in measurable amounts (greater than 1 microgram/mg creatinine). Nor did patients with succinic aciduria excrete succinylglycine in amounts larger than 1 microgram/mg creatinine. On the basis of these data it is argued that production of short- and medium-chain dicarboxylglycines is not a metabolic pathway of biological significance for the elimination of short- and medium-chain dicarboxylic acids from individuals with dicarboxylic acidurias.
D000293:Adolescent; D000328:Adult; D000600:Amino Acids, Dicarboxylic / Q000652:urine*; D002648:Child; D002675:Child, Preschool; D003404:Creatinine / Q000652:urine; D008401:Gas Chromatography-Mass Spectrometry; D005998:Glycine / Q000031:analogs & derivatives* / Q000652:urine; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008314:Malonates / Q000652:urine
D000600:Amino Acids, Dicarboxylic; D008314:Malonates; C015130:N-dicarboxylmonoglycine; D003404:Creatinine; D005998:Glycine