Organic acids or urine in multiple sclerosis.
The urinary excretion of organic acids was examined in 509 cases with multiple sclerosis and in 50 age- and sex-matched controls. The concentrations of the acids were related to creatinine. No differences were found for compounds such as glycolic acid, 2-methyl-3-hydroxybutyric acid, 2-ethylhydracrylic acid, 4-hydroxyphenylacetic acid, suberic acid and many other acids. However, the mean excretion of 2-hydroxy-2-methyl-3-butenoic acid was increased two-fold in the MS group. 2 MS cases had a very high excretion of 3-methylglutaconic acid, and another 6 cases had moderate elevations, which were fairly constant over a time period of several months. Moderate elevations were also noted in 2 healthy controls. 1 MS case had a very high excretion of 3-hydroxyisovaleric acid. 7 MS cases, and none in the control group, had elevated excretion of adipic acid. Differences were also noted for lactic acid, succinic acid, aconitic acid and 3-methyladipic acid. An oral dose of deuterium-labelled acetate was given to one of the patients with high excretion of 3-methylglutaconic acid. Deuterium was incorporated into this metabolite. 3-methylglutaconic acid, 2-hydroxy-2-methyl-3-butenoic acid, 3-hydroxy-isovaleric acid and 3-methyladipic acid are all potential isoprenoid metabolites. A possible defect in the pathway of isoprenoid biosynthesis is discussed.
D000156:Aconitic Acid / Q000652:urine; D000272:Adipates / Q000652:urine; D002264:Carboxylic Acids / Q000652:urine*; D005229:Fatty Acids, Monounsaturated; D005231:Fatty Acids, Unsaturated / Q000652:urine; D005260:Female; D005977:Glutarates / Q000652:urine; D006801:Humans; D007773:Lactates / Q000652:urine; D019344:Lactic Acid; D008297:Male; D009103:Multiple Sclerosis / Q000378:metabolism / Q000652:urine*; D013386:Succinates / Q000652:urine; D019802:Succinic Acid; D013729:Terpenes / Q000378:metabolism; D014631:Valerates / Q000652:urine
D000272:Adipates; D002264:Carboxylic Acids; D005229:Fatty Acids, Monounsaturated; D005231:Fatty Acids, Unsaturated; D005977:Glutarates; D007773:Lactates; D013386:Succinates; D013729:Terpenes; D014631:Valerates; C033274:3-methyladipic acid; C039884:2-hydroxy-2-methyl-3-butenoic acid; D019344:Lactic Acid; C004961:beta-hydroxyisovaleric acid; C022034:3-methylglutaconic acid; C029900:adipic acid; D000156:Aconitic Acid; D019802:Succinic Acid