Journal
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
Volume 1536, Issue 2-3, Pages 133-140Publisher
ELSEVIER
DOI: 10.1016/S0925-4439(01)00041-2
Keywords
chelator; desferrioxamine; Friedreich's ataxia; iron; iron overload disease; pyridoxal isonicotinoyl hydrazone
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Friedreich's ataxia (FA) is a crippling neurodegenerative disease that is due to iron (Fe) overload within the mitochondrion. One therapeutic intervention may be the development of a chelator that could remove mitochondrial Fe. We have implemented the only well characterized model of mammalian mitochondrial Fe overload to examine the Fe chelation efficacy of novel chelators of the 2-pyridylcarboxaldehyde isonicotinoyl hydrazone (PCIH) class. In this model we utilize reticulocytes treated with the haem synthesis inhibitor succinylacetone which results in mitochondrial Fe-loading. Our experiments demonstrate that in contrast to desferrioxamine, several of the PCIH analogues show very high activity at mobilizing Fe-59 from Fe-59-loaded reticulocytes. Further studies on these ligands in animals are clearly warranted considering their potential to treat FA. (C) 2001 Elsevier Science B.V. All rights reserved.
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