Identification and quantitative analysis of urinary organic acids
نویسندگان
چکیده
KODAMA, H., OKADA, S., INUI, K., YUTAKA, T. and YABUUCHI, H. Studies on ƒ¿-Ketoglutaric Aciduria in Type I Glycogenosis. Tohoku J. exp. Med., 1980, 131 (4), 347-353 Urinary excretion of the organic acids in patients with type I and III glycogenosis was investigated. In all patients with type I glycogenosis, urinary ƒ¿-ketoglutarate concentration was about 10 times the normal value. ƒ¿Ketoglutaric aciduria was not improved by the acute or prolonged administration of a large dose of cofactors for pyruvateand ƒ¿-ketoglutarate dehydrogenase complex. On the other hand, the level of ƒ¿-ketoglutarate in the urine from type I patients decreased in conjunction with the decrease of plasma lactate and pyruvate con centration after repeated oral glucose loading. Oral citrate loading brought an increased excretion of ƒ¿-ketoglutarate in type I glycogenosis. It is possible that ƒ¿-ketoglutarate dehydrogenase is the rate-limiting step in tricarboxylic acid cycle
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