Congenital Erythropoietic Protoporphyria : II. An Experimental Study

Investigations into the pathophysiology of erythropoietic protoporphyria suggest that the basic defect is an inborn error of metabolism resulting in the overproduction of protoporphyrin IX by the bone marrow not in response to a failure of heme synthesis. A method is described for evaluating the in vitro synthesis of protoporphyrin and heme by human bone marrow.