Congenital erythropoietic porphyria Other Information

CEP; Erythropoietic Porphyria; Erythropoietic uroporphyria; Guenther porphyria; Gunther Disease; UROS deficiency

This article is about the disease. For the poem by Robert Browning see Porphyria's Lover. The porphyrias are inherited or acquired disorders of certain enzymes in the heme biosynthetic pathway (also called porphyrin pathway). They are broadly classified as hepatic porphyrias or erythropoietic porphyrias, based on the site of the overproduction and mainly accumulation of the porphyrins (or their chemical precursors). Overview In humans, porphyrins are the main precursors of heme, an ...