The accumulation of iron in mitochondria, which is the centre for cell respiration and energy production, is toxic. The iron can substantially damage the cell and cause death.
Using a mouse model, 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 utilization in two major mitochondrial pathways.
“The terrible part is that these children (with high iron accumulation in cells) grow up knowing the joys of self-sufficiency, being able to walk and function normally before they are struck down,” said
The study appeared in the latest edition of the Proceedings of the National Academy of Science.