Citations to this article

Light-induced protoporphyrin release from erythrocytes in erythropoietic protoporphyria.
S Sandberg, A Brun
S Sandberg, A Brun
Published September 1, 1982
Citation Information: J Clin Invest. 1982;70(3):693-698. https://doi.org/10.1172/JCI110664.
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Research Article

Light-induced protoporphyrin release from erythrocytes in erythropoietic protoporphyria.

Abstract

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.

Authors

S Sandberg, A Brun

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Citations to this article (29)

Title and authors Publication Year
In vitro Assessment of Solar Filters for Erythropoietic Protoporphyria in the Action Spectrum of Protoporphyrin IX.
Sernicola A, Cama E, Pelizzo MG, Tessarolo E, Nicolli A, Viero G, Alaibac M 		Frontiers in Medicine 2021
The essential role of the transporter ABCG2 in the pathophysiology of erythropoietic protoporphyria
P Wang, M Sachar, J Lu, AI Shehu, J Zhu, J Chen, K Liu, KE Anderson, W Xie, FJ Gonzalez, CD Klaassen, X Ma 		Science Advances 2019
Protoporphyrin IX in the skin measured noninvasively predicts photosensitivity in patients with erythropoietic protoporphyria
IM Heerfordt, HC Wulf 		The British journal of dermatology 2016
Dual-wavelength excitation for fluorescence-based quantification of zinc protoporphyrin IX and protoporphyrin IX in whole blood
G Hennig, C Gruber, M Vogeser, H Stepp, S Dittmar, R Sroka, GM Brittenham 		Journal of Biophotonics 2013
Leukocytoclastic vasculitis masking chronic vascular changes in previously undiagnosed erythropoietic protoporphyria: Vasculitis in protoporphyria
G Thom, M Lam, M Lucas, E Rossi 		Journal of Cutaneous Pathology 2013
Evolution of the haem synthetic pathway in kinetoplastid flagellates: An essential pathway that is not essential after all?
L Kořený, J Lukeš, M Oborník 		International Journal for Parasitology 2010
DNA-based diagnosis of erythropoietic protoporphyria in two families and the frequency of a low-expression FECH allele in a Chinese population
KC Lau, CW Lam, B Fong, TS Siu, S Tam 		Clinica Chimica Acta 2009
Tetrapyrroles
MJ Warren, AG Smith 		2009
Automated imaging of circulating fluorocytes for the diagnosis of erythropoietic protoporphyria: a pilot study for population screening
KC Lau, CW Lam 		Journal of Medical Screening 2008
Erythrocytes—the ‘house elves’ of photodynamic therapy
L Kaestner, A Juzeniene, J Moan 		Photochemical & Photobiological Sciences 2004
The Porphyrin Handbook
PM Shoolingin-Jordan 		The Porphyrin Handbook 2003
Effects of photoproducts on the binding properties of protoporphyrin IX to proteins
L Brancaleon, H Moseley 		Biophysical Chemistry 2002
Genetic Rescue of Leishmania Deficiency in Porphyrin Biosynthesis Creates Mutants Suitable for Analysis of Cellular Events in Uroporphyria and for Photodynamic Therapy
JF Sah, H Ito, BK Kolli, DA Peterson, S Sassa, KP Chang 		The Journal of biological chemistry 2002
Porphyrins, porphyrin metabolism and porphyrias. IV. Pathophysiology of erythyropoietic protoporphyria - diagnosis, care and monitoring of the patient
ST Brun 		Scandinavian Journal of Clinical & Laboratory Investigation 2000
Protoporphyrin IX and oxidative stress
S Afonso, G Vanore, A Batlle 		Free Radical Research 1999
The photodynamic and non-photodynamic actions of porphyrins
SG Afonso, RE de Salamanca, AM Batlle 		Brazilian journal of medical and biological research = Revista brasileira de pesquisas medicas e biologicas / Sociedade Brasileira de Biofisica ... [et al.] 1999
Folding and unfolding of δ-aminolevulinic acid dehydratase and porphobilinogen deaminase induced by uro- and protoporphyrin
SG Afonso, RE de Salamanca, A Batlle 		The International Journal of Biochemistry & Cell Biology 1997
Porphyrin-induced protein structural alterations of heme enzymes
SG Afonso, RE de Salamanca, AM Batlle 		The International Journal of Biochemistry & Cell Biology 1997
Comparison of the photobleaching effect of three photosensitizing agents: Meso-tetra(m-hydroxyphenyl)chlorin, meso-tetra(m-hydroxyphenyl)porphyrin and photofrin during photodynamic therapy
LW Ma, J Moan, K Berg 		Lasers in Medical Science 1994
Accumulation of iron in erythroblasts of patients with erythropoietic protoporphyria
LH Rademakers, JC Koningsberger, CW Sorber, HB de la Faille, JV Hattum, JJ Marx 		European Journal of Clinical Investigation 1993
Hemolytic anemia in protoporphyria: Possible precipitating role of liver failure and photic stress
NS Key, JM Rank, D Freese, JR Bloomer, DE Hammerschmidt 		American Journal of Hematology 1992
Mechanisms of photosensitivity in porphyric patients with special emphasis on erythropoietic protoporphyria
A Brun, S Sandberg 		Journal of Photochemistry and Photobiology B Biology 1991
ERYTHROPOIETIC PROTOPORPHYRIA: PHOTODYNAMIC TRANSFER OF PROTOPORPHYRIN FROM INTACT ERYTHROCYTES TO OTHER CELLS
A Brun, A Western, Z Malik, S Sandberg 		Photochemistry and Photobiology 1990
A change in the quantum yield of photoinactivation of cells observed during photodynamic treatment
J Moan 		Lasers in Medical Science 1988
Light-induced redistribution and photobleaching of protoporphyrin in erythrocytes from patients with erythropoietic protoporphyria: An explanation of the rapid fading of fluorocytes
A Brun, S Sandberg 		Journal of Photochemistry and Photobiology B Biology 1988
Photoproducts formed from photofrin II in cells
J Moan, D Kessel 		Journal of Photochemistry and Photobiology B: Biology 1988
Photosensitisation
G Moreno, RH Pottier, TG Truscott 		1988
Toxic and Phototoxic Effects of Tetraphenylporphinesulphonate and Haematoporphyrin Derivative in Vitro
JF Evensen, J Moan, JW Winkelman 		International Journal of Radiation Biology and Related Studies in Physics, Chemistry and Medicine 1987
PHOTODYNAMIC RELEASE OF PROTOPORPHYRIN FROM INTACT ERYTHROCYTES IN ERYTHROPOIETIC PROTOPORPHYRIA: THE EFFECT OF SMALL REPETITIVE LIGHT DOSES
A Brun, S Sandberg 		Photochemistry and Photobiology 1985

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