Showing papers on "Protoporphyrin IX published in 1967"
TL;DR: A direct relationship between the α-helix content of myoglobin derivatives as determined by optical activity and the number of normally titratable imidazole side chains is supported.
50 citations
TL;DR: Increased numbers of red-fluorescing erythrocytes with normal RBC protoporphyrin concentrations in four asymptomatic relatives of two patients with EPP are reported.
Abstract: IN 1953, Kosenow and Treibs 1 described a new inborn error of porphyrin metabolism which Magnus et al 2 later termed erythropoietic protoporphyria (EPP). Porter provided evidence that EPP was due to overproduction of protoporphyrin IX in the bone marrow. 3 Erythrocyte and fecal protoporphyrins and, at times, coproporphyrins are increased, but urinary porphyrins are normal in affected individuals. 1 Erythrocytes 1,4,5 and their precursors 6,7 have been observed to fluoresce in some cases but not in others. 8 A dominant form of inheritance has been suggested by several authors. 6,9-12 Asymptomatic relatives with "latent" EPP have been previously identified by slightly increased red blood cell (RBC) protoporphyrin concentrations. 6,13,14 We now report increased numbers of red-fluorescing erythrocytes with normal RBC protoporphyrin concentrations in four asymptomatic relatives of two patients with EPP. In addition, a sibling of one of the patients was found to have elevated RBC protoporphyrin levels and
8 citations
7 citations
1 citations