Porphyrin formation and hemoglobin metabolism in congenital porphyria.

The studies reported here were conducted on a patient with congenital porphyria who excretes large amounts of uroporphyrin I and coproporphyrin I. They were directed to the following problems: (a) to determine whether glycine is specifically utilized in the biologic synthesis of porphyrins related to the etioporphyrin I configuration as it is utilized in the synthesis of protoporphyrin which is related to the etioporphyrin III configuration (1) ; (b) to measure the rates of formation (and degradation) or uroporphyrin I and coproporphyrin I; (c) to investigate the life span and pattern of destruction of the erythrocyte and the origins of bile pigment in this disease.