Publication year
2013Source
Nederlands Tijdschrift voor Geneeskunde, 157, 49, (2013)-A6258ISSN
Publication type
Article / Letter to editor
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Organization
Pathology
Laboratory of Genetic, Endocrine and Metabolic Diseases
Journal title
Nederlands Tijdschrift voor Geneeskunde
Volume
vol. 157
Issue
iss. 49
Page end
p. A6258
Subject
IGMD 7: Iron metabolism; IGMD 7: Iron metabolism N4i 1: Pathogenesis and modulation of inflammationAbstract
- Iron overload disorders are common and, if left untreated, severe systemic diseases that can have both genetic and acquired causes.- Hereditary haemochromatosis, beta-thalassaemia, myelodysplastic syndromes and sickle cell disease are among the most important examples.- Iron that is not bound to transferrin, haem or ferritin (non-transferrin-bound iron, NTBI) seems to play a key role in the pathophysiology of these disorders.- NTBI is a heterogeneous group of potentially toxic iron complexes in plasma which are generated almost exclusively under pathological conditions.- Cellular uptake of NTBI contributes to its toxicity and is mediated by several organ-specific transporters and receptors.- NTBI-induced toxicity is the result of oxidative damage to various macromolecules by reactive oxygen species (ROS).- In the near future, we hypothesize that NTBI will have important implications for both diagnosis and treatment of iron overload disorders.- However, before NTBI can be applied to patient care, the currently available assays need further clinical and analytical validation.
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- Faculty of Medical Sciences [90373]
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