title: Fluorescent cytoplasm and Heinz bodies of hemoglobin Köln erythrocytes: evidence for intracellular heme catabolism.
abstract: Hb Köln, one of the common mutant hemoglobins responsible for unstable hemoglobin disease, was found to be degraded to a fluorescent yellow pigment (FYP) in circulating erythrocytes. FYP is responsible for a strong green fluorescence observed in the cytoplasm and is particularly abundant in the Heinz bodies of Köln RBC. Front face fluorometry and fluorescence microscopy showed that Heinz bodies emit 10% to 20% of the fluorescence of RBCs. Hb-free FYP was obtained by means of a cellulose column separation of the cytoplasm or from a precipitate formed during the incubation of Köln RBC cytoplasm at 50 degrees C. The absorption and emission spectra of FYP are consistent with those of dipyrroles.
mesh_terms: D000328:Adult; D002648:Child; D004913:Erythrocytes, Abnormal / Q000378:metabolism*; D005260:Female; D005453:Fluorescence; D006366:Heinz Bodies / Q000378:metabolism; D006418:Heme / Q000378:metabolism*; D006455:Hemoglobins, Abnormal / Q000378:metabolism*; D006801:Humans; D008297:Male; D008856:Microscopy, Fluorescence; D010860:Pigments, Biological / Q000096:biosynthesis
chemical_list: D006455:Hemoglobins, Abnormal; D010860:Pigments, Biological; D006418:Heme; C034409:hemoglobin Koln