Subject(s)
Chloroquine/pharmacology , Porphyrias/drug therapy , Skin Manifestations , 5-Aminolevulinate Synthetase/metabolism , Animals , Chemical Phenomena , Chemistry , Chloroquine/therapeutic use , Chromatography, Gel , Disease Models, Animal , Female , Hydrogen-Ion Concentration , Liver/analysis , Liver/enzymology , Liver/pathology , Mitochondria, Liver/metabolism , Oxidative Phosphorylation , Porphyrias/metabolism , Porphyrias/pathology , Porphyrins/analysis , Porphyrins/blood , Porphyrins/urine , Primaquine/pharmacology , Primaquine/therapeutic use , Rats , Spectrophotometry, AtomicSubject(s)
Carcinoma, Ehrlich Tumor/metabolism , Dextrans/pharmacology , Glycolysis/drug effects , Adenine Nucleotides/metabolism , Adenosine Triphosphatases/metabolism , Aerobiosis , Animals , Antifungal Agents/pharmacology , Biological Transport, Active/drug effects , Carcinoma, Ehrlich Tumor/enzymology , Cell Membrane Permeability/drug effects , Dextrans/antagonists & inhibitors , Dinitrophenols/pharmacology , Lactates/metabolism , Mice , Mitochondria/metabolism , Ouabain/pharmacology , Oxygen , Phosphorus Isotopes , Potassium/metabolism , Potassium/pharmacology , Sodium/metabolism , Sodium/pharmacology , Sulfuric Acids/pharmacologySubject(s)
Acyltransferases , Heme , Liver/enzymology , 5-Aminolevulinate Synthetase/antagonists & inhibitors , Animals , Binding Sites , Carbon Isotopes , Coenzyme A , Cytoplasm/enzymology , Ethers , Feedback , Glycine , Kinetics , Male , Models, Chemical , Porphyrins , Pyridoxal Phosphate , Rats , Schiff Bases , Solubility , Structure-Activity Relationship , Succinates , Sulfhydryl Compounds , Sulfhydryl ReagentsSubject(s)
Acyltransferases/isolation & purification , Liver/enzymology , 5-Aminolevulinate Synthetase/biosynthesis , 5-Aminolevulinate Synthetase/isolation & purification , Acetamides/pharmacology , Allyl Compounds/pharmacology , Ammonium Sulfate , Animals , Calcium Phosphates , Chromatography, Gel , Cytoplasm/enzymology , Enzyme Activation , Enzyme Induction , Hydrogen-Ion Concentration , Immunodiffusion , Kinetics , Liver/cytology , Male , Molecular Weight , Protamines , Rabbits/immunology , Rats , Solubility , Structure-Activity Relationship , UltracentrifugationABSTRACT
A patient with erythropoietic protoporphyria was studied to determine the sites of excess protoporphyrin formation. The patient's protoporphyrin was pulse labeled by the simultaneous administration of the precursors 2-glycine-(14)C and 3,5-delta-aminolevulinic acid-(3)H; delta-aminolevulinic acid preferentially labels the hepatic pool. Blood and feces were studied at intervals for the next 14 days. Protoporphyrin was extracted from erythrocytes, plasma, and feces, identified by thin-layer chromatography, and quantitated spectrophotometrically, and its specific activity was determined by liquid scintillation spectrometry. Analysis of the kinetic and isotopic data indicated at least two sources of protoporphyrin, one localized in the erythroid cells, a second in the liver. The liver was responsible for the majority of the excess protoporphyrin. This report thus provides evidence of a genetic porphyria exhibiting an abnormality of porphyrin biosynthesis in at least two tissues. We propose that the disease, erythropoietic protoporphyria, be renamed erythrohepatic protoporphyria.
