Build-up of iron in cells causes debilitating disease

SYDNEY - The build-up of iron in cellular mitochondria, the centre for cell respiration and energy production, can bring on a debilitating hereditary disease like Friedreich’s Ataxia (FA). The disease causes brain and cardiac disorders, ending in early death.

The accumulation of iron in mitochondria is toxic, substantially damaging the cell and causing death.

Using a mouse model of FA, University of Sydney (U-S) researchers found that the iron loading was caused by its increased iron uptake and decreased release due to reduced iron utilisation in two major mitochondrial pathways.

“The terrible part is that these children grow up knowing the joys of self-sufficiency, being able to walk and function normally before they are struck down,” said Des Richardson.

Michael Huang, study co-author noted: “It’s great to work on such an intractable disease and by unveiling its underlying nuts and bolts to get results that can potentially help lots of people.”

The study appeared in the latest edition of The Proceedings of the National Academy of Science.