Iron homeostasis during transfusional iron overload in β-thalassemia and sickle cell disease: Changes in iron regulatory protein, hepcidin, and ferritin expression

Zandra A. Jenkins, Ward Hagar, Christopher Bowlus, Hans E. Johansson, Paul Harmatz, Elliott P. Vichinsky, Elizabeth C. Theil

Research output: Contribution to journalArticle

25 Scopus citations

Abstract

□ Hypertransfusional (>8 transfusions/year) iron in liver biopsies collected immediately after transfusions in β-thalassemia and sickle cell disease correlated with increased expression (RNA) for iron regulatory proteins 1 and 2 (3-, 9- to 11-fold) and hepcidin RNA: (5- to 8-fold) (each p <.01), while ferritin H and L RNA remained constant. A different H:L ferritin ratio in RNA (0.03) and protein (0.2-0.6) indicated disease-specific trends and suggests novel post-transcriptional effects. Increased iron regulatory proteins could stabilize the transferrin receptor mRNA and, thereby, iron uptake. Increased hepcidin, after correction of anemia by transfusion, likely reflects excess liver iron. Finally, the absence of a detectable change in ferritin mRNA indicates insufficient oxidative stress to significantly activate MARE/ARE promoters.

Original languageEnglish (US)
Pages (from-to)237-243
Number of pages7
JournalPediatric Hematology and Oncology
Volume24
Issue number4
DOIs
StatePublished - May 2007

Keywords

  • β-thalassemia
  • Ferritin
  • Hepcidin
  • Hypertransfusion iron overload
  • Iron regulatory proteins
  • Sickle cell disease

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology
  • Cancer Research

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