The pathogenic mutation in Friedreich's ataxia (FRDA) is a homozygous guanine-adenine-
adenine (GAA) trinucleotide repeat expansion on chromosome 9q13 that causes a …

Maintenance of proper “labile iron” levels is a critical component in preserving homeostasis.
Iron is a vital element that is a constituent of a number of important macromolecules …

Iron and oxygen share a delicate partnership since both are indispensable for survival, but if
the partnership becomes inadequate, this may rapidly terminate life. Virtually all cell …

Work during the past 14 years has shown that mitochondria are the primary site for the
biosynthesis of iron–sulfur (Fe/S) clusters. In fact, it is this process that renders mitochondria …

Abstract Iron–sulfur (Fe–S) clusters are ancient, ubiquitous cofactors composed of iron and
inorganic sulfur. The combination of the chemical reactivity of iron and sulfur, together with …

Genetic disorders of iron metabolism and chronic inflammation often evoke local iron
accumulation. In Friedreich ataxia, decreased iron-sulphur cluster and heme formation leads …

Friedreich ataxia (FRDA) is an autosomal-recessive disorder characterized by progressive
neurological and cardiac abnormalities. It predominantly affects individuals of European and …

Understanding the role of frataxin in mitochondria is key to an understanding of the
pathogenesis of Friedreich ataxia. Frataxins are small essential proteins whose deficiency …

Friedreich ataxia (FRDA) is a neurodegenerative disorder caused by an unstable GAA
repeat expansion mutation within intron 1 of the FXN gene. However, the origins of the GAA …

Two groups of hereditary ataxias are most relevant to humans—the autosomal recessive
ataxias and the autosomal dominant spinocerebellar ataxias. Recessive ataxias are …