The ins and outs of mitochondrial iron-loading: the metabolic defect in Friedreich's ataxia.

J Mol Med. 2009 Dec 9.

Richardson DR, Huang ML, Whitnall M, Becker EM, Ponka P, Suryo Rahmanto Y.


Iron Metabolism and Chelation Program, Department of Pathology and Bosch Institute, Blackburn Building (D06), University of Sydney, Sydney, NSW, 2006, Australia, d.richardson@med.usyd.edu.au.

Keywords: Friedreich's ataxia, cardio- and neurodegenerative disease, frataxin, mitochondrial iron-overload, transferrin receptor-1 upregulation, ferritin, ferroportin1, mitoferrin2, iron-sulfur cluster (ISC), mitochondrial ferritin, compensatory alterations.