Abstract
Abstract The metabolic products of propionate metabolism in man were studied by determining the pattern of isotope in the urine following the intravenous injection of [1-14C]propionate. A major peak of radioactivity not present in control subjects was found in an organic acid in two patients with propionic acidemia and a patient with methylmalonic acidemia. This labeled compound was characterized as methylcitrate. Methylcitrate excretion was appreciable in patients with defective propionic acid metabolism and could be detected without administration of isotope. Methylcitrate is considered to be formed from the condensation of propionyl-CoA and oxalacetate. It could be involved in the pathogenesis of the clinical manifestations of these conditions.
Highlights
Methylcitrate excretion was appreciable in patients with defective propionic acid metabolism and could be detected without administration of isotope
A study of the metabolism of propionate was undertaken in order to elucidate t,he clinical and chemical manifest.ations of patients with propionic acidemia and methylmalonic acidemia
In two other patients with propionic, acidemia, K.11. arid J.R., urine samples bvere obtained for the :trlalysis of nonisotopic compounds escret,ed
Summary
The metabolic products of propionate metabolism in man were studied by determining the pattern of isotope in the urine following the intravenous injection of [lJ%]propionate. A major peak of radioactivity not present in control subjects was found in an organic acid in two patients with propionic acidemia and a patient with methylmalonic acidemia. This labeled compound was characterized as methylcitrate. Methylcitrate excretion was appreciable in patients with defective propionic acid metabolism and could be detected without administration of isotope. Methylcitrate is considered to be formed from the condensation of propionyl-CoA and oxalacetate It could be involved in the pathogenesis of the clinical manifestations of these conditions. A study of the metabolism of propionate was undertaken in order to elucidate t,he clinical and chemical manifest.ations of patients with propionic acidemia and methylmalonic acidemia.
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
