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Characterization of protoporphyrin in red blood cells of patients with erythropoietic protoporphyria.
Clin Chim Acta. 1975 Sep 16; 63(3):355-62.CC

Abstract

It was investigated whether the protoporphyrin that can be extracted from red blood cells of erythropoietic protoporphyria (E.P.P.) patients is present in the cells as free molecules or protein-bound. With isoelectric focusing and with starch gel electrophoresis it could be shown that virtually all protoporphyrin in the erythrocytes is protein-bound. It is very likely that the protoporphyrin is bound to hemoglobin at heme-binding sites. This was indicated by several observations: 1. With isoelectric focusing the protoporphyrin-protein complex is focused at a pH only slightly higher than the isoelectric point of hemoglobin. 2. With chromatography on Sephadex columns it appeared that hemoglobin and the protopotphyrin-protein complex have the same molecular weight. 3. A Heme-protoporphyrin exchange occurred when the heme-globin bond was labialized by conversion to hemiglobin. The resulting protoporphyrin-hemoglobin complex had the same electrophoretic mobility with starch gel electrophoresis as the protoporphyrin-protein complex, extracted from red blood cells of E.P.P. patients.

Authors

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Pub Type(s)

Journal Article

Language

eng

PubMed ID

1175305

Citation

De Goeij, A F., et al. "Characterization of Protoporphyrin in Red Blood Cells of Patients With Erythropoietic Protoporphyria." Clinica Chimica Acta; International Journal of Clinical Chemistry, vol. 63, no. 3, 1975, pp. 355-62.
De Goeij AF, Van Steveninck J, Went LN. Characterization of protoporphyrin in red blood cells of patients with erythropoietic protoporphyria. Clin Chim Acta. 1975;63(3):355-62.
De Goeij, A. F., Van Steveninck, J., & Went, L. N. (1975). Characterization of protoporphyrin in red blood cells of patients with erythropoietic protoporphyria. Clinica Chimica Acta; International Journal of Clinical Chemistry, 63(3), 355-62.
De Goeij AF, Van Steveninck J, Went LN. Characterization of Protoporphyrin in Red Blood Cells of Patients With Erythropoietic Protoporphyria. Clin Chim Acta. 1975 Sep 16;63(3):355-62. PubMed PMID: 1175305.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Characterization of protoporphyrin in red blood cells of patients with erythropoietic protoporphyria. AU - De Goeij,A F, AU - Van Steveninck,J, AU - Went,L N, PY - 1975/9/16/pubmed PY - 1975/9/16/medline PY - 1975/9/16/entrez SP - 355 EP - 62 JF - Clinica chimica acta; international journal of clinical chemistry JO - Clin Chim Acta VL - 63 IS - 3 N2 - It was investigated whether the protoporphyrin that can be extracted from red blood cells of erythropoietic protoporphyria (E.P.P.) patients is present in the cells as free molecules or protein-bound. With isoelectric focusing and with starch gel electrophoresis it could be shown that virtually all protoporphyrin in the erythrocytes is protein-bound. It is very likely that the protoporphyrin is bound to hemoglobin at heme-binding sites. This was indicated by several observations: 1. With isoelectric focusing the protoporphyrin-protein complex is focused at a pH only slightly higher than the isoelectric point of hemoglobin. 2. With chromatography on Sephadex columns it appeared that hemoglobin and the protopotphyrin-protein complex have the same molecular weight. 3. A Heme-protoporphyrin exchange occurred when the heme-globin bond was labialized by conversion to hemiglobin. The resulting protoporphyrin-hemoglobin complex had the same electrophoretic mobility with starch gel electrophoresis as the protoporphyrin-protein complex, extracted from red blood cells of E.P.P. patients. SN - 0009-8981 UR - https://www.unboundmedicine.com/medline/citation/1175305/Characterization_of_protoporphyrin_in_red_blood_cells_of_patients_with_erythropoietic_protoporphyria_ L2 - https://linkinghub.elsevier.com/retrieve/pii/0009-8981(75)90057-1 DB - PRIME DP - Unbound Medicine ER -