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 …

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 …

Expansion of GAA· TTC triplets within an intron in FXN (the gene encoding frataxin) leads to
transcription silencing, forming the molecular basis for the neurodegenerative disease …

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

In its classic hormonal role, erythropoietin (EPO) is produced by the kidney and regulates
the number of erythrocytes within the circulation to provide adequate tissue oxygenation …

The predominant molecular symptom of aging is the accumulation of altered gene products.
Moreover, several conditions including protein, lipid or glucose oxidation disrupt redox …

Autosomal-recessive cerebellar ataxias (ARCAs) comprise a heterogeneous group of rare
degenerative and metabolic genetic diseases that share the hallmark of progressive …

Friedreich ataxia is an autosomal recessive degenerative disease that primarily affects the
nervous system and the heart. It is named after its original description as a “degenerative …

The study investigates if alpha-lipoic acid is neuroprotective against chemotherapy induced
neurotoxicity, if mitochondrial damage plays a critical role in toxic neurodegenerative …

Mitochondrial dysfunction and oxidative damage are at the origin of numerous
neurodegenerative diseases like Friedreich ataxia and Alzheimer and Parkinson diseases …