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Light-induced protoporphyrin release from erythrocytes in erythropoietic protoporphyria

Journal Article · · J. Clin. Invest.; (United States)
DOI:https://doi.org/10.1172/JCI110664· OSTI ID:6564767
;
The photohemolysis of normal erythrocytes incubated with protoporphyrin is reduced in the presence of albumin. When globin is added to normal erythrocytes loaded with protoporphyrin, protoporphyrin is bound to globin. During irradiation protoporphyrin moves from globin to the erythrocyte membrane and photohemolysis is initiated. Erythrocytes in patients with erythropoietic protoporphyria contain large amounts of protoporphyrin bound to hemoglobin. Upon irradiation of these cells in the absence of albumin, 40% of protoporphyrin and 80% of hemoglobin is released after 240 kJ/m2. The released protoporphyrin is hemoglobin bound. In contrast, when albumin is present only 8% of hemoglobin is released whereas protoporphyrin is released to 76%. The released protoporphyrin is albumin bound. A hypothesis for the release of erythrocyte protoporphyrin in erythropoietic protoporphyria without simultaneous hemolysis is proposed. Upon irradiation protoporphyrin photodamages its binding sites on hemoglobin, moves through the plasma membrane, and is bound to albumin in plasma.
Research Organization:
Department of Dermatology, University of Bergen, Sykehus, Norway
OSTI ID:
6564767
Journal Information:
J. Clin. Invest.; (United States), Journal Name: J. Clin. Invest.; (United States) Vol. 70:3; ISSN JCINA
Country of Publication:
United States
Language:
English

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Related Subjects

560115* -- Radiation Effects on Biochemicals-- In Man-- (-1987)
560151 -- Radiation Effects on Animals-- Man
63 RADIATION, THERMAL, AND OTHER ENVIRON. POLLUTANT EFFECTS ON LIVING ORGS. AND BIOL. MAT.
ALBUMINS
BIOCHEMISTRY
BIOLOGICAL EFFECTS
BIOLOGICAL MATERIALS
BIOLOGICAL PATHWAYS
BIOLOGICAL RADIATION EFFECTS
BLOOD
BLOOD CELLS
BODY FLUIDS
CARBOXYLIC ACIDS
CELL CONSTITUENTS
CELL MEMBRANES
CHEMISTRY
ERYTHROCYTES
EXTERNAL IRRADIATION
GLOBIN
HEMOGLOBIN
HEMOLYSIS
HETEROCYCLIC ACIDS
HETEROCYCLIC COMPOUNDS
IRRADIATION
LYSIS
MATERIALS
MEDICINE
MEMBRANES
NUCLEAR MEDICINE
ORGANIC ACIDS
ORGANIC COMPOUNDS
ORGANIC NITROGEN COMPOUNDS
PATHOLOGICAL CHANGES
PATIENTS
PIGMENTS
PORPHYRINS
PROTEINS
PROTOPORPHYRINS
RADIATION EFFECTS
RADIOLOGY
RADIOTHERAPY
THERAPY