Friedreich's ataxia is a neurodegenerative disease caused by a decrease in the mitochondrial protein frataxin.  Frataxin, an iron(II) chaperone, delivers the metal to proteins which require iron for their function.  Decreased frataxin levels result in increased free iron within the mitochondria, which through Fenton chemistry promotes oxidative damage to heart and muscle tissue. Despite many efforts, iron coordination by frataxin remains unclear and the experiments to address it are often contradictory.  We have used NMR to study the specific amino acids in frataxin that are binding iron.  We have determined the amino acids that are involved in binding of iron by evaluating both line broadening and chemical shift perturbations.  The results from these studies will be presented and discussed.

Using NMR to determine Fe(II) metal coordination to frataxin

245th National Meeting (2013)

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