Oxidative DNA damage is an inevitable consequence of cellular metabolism, with a
propensity for increased levels following toxic insult. Although more than 20 base lesions …

The brain and nervous system are prone to oxidative stress, and are inadequately equipped
with antioxidant defense systems to prevent 'ongoing'oxidative damage, let alone the extra …

The generation of reactive oxygen species may be both beneficial to cells, performing a
function in inter-and intracellular signalling, and detrimental, modifying cellular …

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 …

Mitochondrial diseases (encephalomyopathies) have traditionally been ascribed to defects
of the respiratory chain, which has helped researchers explain their genetic and clinical …

In recent years, it has become increasingly clear that mitochondrial dysfunction and
oxidative damage are major contributors to neuronal loss. Free radicals, typically generated …

Friedreich ataxia is the most common of the hereditary ataxias. It is due to homozygous/
compound heterozygous mutations in FXN. This gene encodes frataxin, a protein largely …

There is significant evidence that the pathogenesis of several neurodegenerative diseases,
including Parkinson's disease, Alzheimer's disease, Friedreich's ataxia (FRDA), multiple …

Friedreich ataxia (FA) represents the most frequent type of inherited ataxia. Most patients
carry homozygous GAA expansions in the first intron of the frataxin gene on chromosome 9 …

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 …