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Decreased Haem Synthetase Activity in Blood Cells of Patients with Erythropoietic Protoporphyria
Author(s) -
Goeij A. F. P. M.,
Christianse K.,
Steveninck J.
Publication year - 1975
Publication title -
european journal of clinical investigation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.164
H-Index - 107
eISSN - 1365-2362
pISSN - 0014-2972
DOI - 10.1111/j.1365-2362.1975.tb00470.x
Subject(s) - erythropoietic protoporphyria , protoporphyrin , medicine , red blood cell , endocrinology , protoporphyrin ix , chemistry , heme , enzyme , ferrochelatase , erythropoiesis , cell , biochemistry , biology , anemia , porphyrin , photodynamic therapy , organic chemistry
. A constant finding in erythropoietic protoporphyria (E. P. P.) is a raised protoporphyrin content of the erythrocytes. It has been shown before that the biosynthesis of this protoporphyrin takes place in the erythroid cell. In circulating blood cells of patients with E. P. P. the haem synthetase activity is significantly decreased. This observation and the increased protoporphyrin concentration in the cells can be explained by assuming a decreased stability of haem synthetase in the red blood cells of E. P. P. patients.