A study of the porphyrins excreted in the urine by a case of congenital porphyria

Abstract

A chromatographic investigation was made of the porphyrins present in the urine of a case of congenital porphyria. The pure uroporphyrin I octamethyl ester isolated represented 77% of the total porphyrin of the urine. Fractions were also isolated behaving as entities on the chromatogram and consisting of 8-carboxyl porphyrins only. Decarboxylation yielded coproporphyrins I and HI; hence these fractions are regarded as associations (possibly molecular) of uroporphyrins I and III. The view is held that in mixtures of esters of the 2 isomers these associations will form and separate chromatographically from the remaining isomer, whichever happens to be in excess. Reports from previous investigations were examined in the light of this suggestion. Small quantities were also isolated of coproporphyrin I and of a 7-carboxyl porphyrin. A 5-carboxyl porphyrin was detected, but was not completely separated from accompanying coproporphyrin.