scholarly journals Ultraviolet A phototest positivity is associated with higher free erythrocyte protoporphyrin IX concentration and lower transferrin saturation values in erythropoietic protoporphyria

2021 ◽  
Author(s):  
Giovanni Genovese ◽  
Carlo Alberto Maronese ◽  
Chiara Moltrasio ◽  
Roberta Piccinno ◽  
Dario Antonio Marletta ◽  
...  
Keyword(s):  
Transferrin Saturation ◽  
Protoporphyrin Ix ◽  
Ultraviolet A ◽  
Erythropoietic Protoporphyria ◽  
Free Erythrocyte Protoporphyrin ◽  
Erythrocyte Protoporphyrin

Erythropoiesis and iron metabolism in dominant erythropoietic protoporphyria

Blood ◽  
2007 ◽  
Vol 110 (12) ◽  
pp. 4108-4110 ◽  
Author(s):  
S. Alexander Holme ◽  
Mark Worwood ◽  
Alexander V. Anstey ◽  
George H. Elder ◽  
Michael N. Badminton
Keyword(s):  
Binding Capacity ◽  
Transferrin Saturation ◽  
Protoporphyrin Ix ◽  
Microcytic Anemia ◽  
Normal Serum ◽  
World Health ◽  
Erythropoietic Protoporphyria ◽  
Iron Supply ◽  
Health Organization ◽  
Iron Binding Capacity

AbstractErythropoietic protoporphyria (EPP) results from deficiency of ferrochelatase (FECH). Accumulation of protoporphyrin IX causes life-long acute photosensitivity. Microcytic anemia occurs in 20% to 60% of patients. We investigated 178 patients with dominant EPP confirmed by molecular analysis. Erythropoiesis was impaired in all patients; all had a downward shift in hemoglobin (Hb), and the mean decreased in males by 12 g/L (1.2 g/dL). By World Health Organization criteria, 48% of women and 33% of men were anemic. Iron stores, assessed by serum ferritin (sFn), were decreased by two-thirds, but normal serum soluble transferrin receptor-1 and iron concentrations suggested that erythropoiesis was not limited by iron supply. FECH deficiency in EPP appears to lead to a steady state in which decreased erythropoiesis is matched by reduced iron absorption and supply. This response may in part be mediated by protoporphyrin, but we found no correlation between erythrocyte protoporphyrin and Hb, sFn, total iron-binding capacity, or transferrin saturation.

scholarly journals Uptake of protoporphyrin IX by isolated rat liver mitochondria

1980 ◽  
Vol 188 (2) ◽  
pp. 329-335 ◽  
Author(s):  
M E Koller ◽  
I Romslo
Keyword(s):  
Rat Liver ◽  
Mitochondrial Membrane ◽  
Protoporphyrin Ix ◽  
Liver Mitochondria ◽  
Rat Liver Mitochondria ◽  
Inner Mitochondrial Membrane ◽  
Erythropoietic Protoporphyria ◽  
Inner Membrane ◽  
Isolated Rat Liver ◽  
Isolated Rat

Rat liver mitochondria accumulate protoporphyrin IX from the suspending medium into the inner membrane in parallel with the magnitude of the transmembrane K+ gradient (K+in/K+out). Only protoporphyrin IX taken up in parallel with the transmembrane K+ gradient is available for haem synthesis. Coproporphyrins (isomers I and III) are not taken up by the mitochondria. The results support the suggestion by Elder & Evans [(1978) Biochem. J. 172, 345-347] that the prophyrin to be taken up by the inner mitochondrial membrane belongs to the protoporphyrin(ogen) IX series. Protoporphyrin IX at concentrations above 15 nmol/mg of protein has detrimental effects on the structural and functional integrity of the mitochondria. The relevance of these effects to the hepatic lesion in erythropoietic protoporphyria is discussed.

Erythrocyte Protoporphyrin IX as a Diagnostic and Therapy Evaluating Tool in Lead Poisoning

1981 ◽  
Vol 36 (1) ◽  
pp. 40-43 ◽  
Author(s):  
MIGUEL ANGEL ZÚÑIGA-CHARLES ◽  
J. DIEGO GONZÁLEZ-RAMÍREZ ◽  
GILBERTO MOLINA-BALLESTEROS
Keyword(s):  
Lead Poisoning ◽  
Protoporphyrin Ix ◽  
Erythrocyte Protoporphyrin

Normative Data of Hemoglobin Concentration and Free Erythrocyte Protoporphyrin in a Private Pediatric Practice

1986 ◽  
Vol 25 (4) ◽  
pp. 206-208 ◽  
Author(s):  
John T. Benjamin ◽  
Michael D. Dickens ◽  
Raymond F. Ford ◽  
Charles H. Gleason ◽  
Vito A. Perriello ◽  
...  
Keyword(s):  
Normative Data ◽  
Hemoglobin Concentration ◽  
Pediatric Practice ◽  
Free Erythrocyte Protoporphyrin ◽  
Erythrocyte Protoporphyrin

Comparison of unitages for total free erythrocyte protoporphyrin (total EP) in anemia screening

1989 ◽  
Vol 180 (1) ◽  
pp. 87-92 ◽  
Author(s):  
Koichi Harada ◽  
Shoko Omori ◽  
Tatsuro Ueno ◽  
Junichi Misumi ◽  
Hajime Miura
Keyword(s):  
Free Erythrocyte Protoporphyrin ◽  
Erythrocyte Protoporphyrin
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