pyridoxal-isonicotinoyl-hydrazone and Friedreich-Ataxia

pyridoxal-isonicotinoyl-hydrazone has been researched along with Friedreich-Ataxia* in 1 studies

Other Studies

1 other study(ies) available for pyridoxal-isonicotinoyl-hydrazone and Friedreich-Ataxia

Article	Year
Development of potential iron chelators for the treatment of Friedreich's ataxia: ligands that mobilize mitochondrial iron. Biochimica et biophysica acta, 2001, May-31, Volume: 1536, Issue:2-3 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 (59)Fe from (59)Fe-loaded reticulocytes. Further studies on these ligands in animals are clearly warranted considering their potential to treat FA. Topics: Animals; Drug Design; Friedreich Ataxia; Iron; Iron Chelating Agents; Iron Radioisotopes; Isoniazid; Ligands; Mice; Mitochondria; Molecular Structure; Phenylhydrazines; Pyridoxal; Reticulocytes	2001

Article

Year

Development of potential iron chelators for the treatment of Friedreich's ataxia: ligands that mobilize mitochondrial iron.

Biochimica et biophysica acta, 2001, May-31, Volume: 1536, Issue:2-3

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 (59)Fe from (59)Fe-loaded reticulocytes. Further studies on these ligands in animals are clearly warranted considering their potential to treat FA.

Topics: Animals; Drug Design; Friedreich Ataxia; Iron; Iron Chelating Agents; Iron Radioisotopes; Isoniazid; Ligands; Mice; Mitochondria; Molecular Structure; Phenylhydrazines; Pyridoxal; Reticulocytes

2001