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Mitochondrial dysfunction and oxidative damage are at the origin of numerous neurodegenerative diseases like Friedreich ataxia and Alzheimer and Parkinson diseases. Friedreich ataxia FRDA is the most common hereditary ataxia, with one individual affected in 50, This disease is characterized by progressive degeneration of the central and peripheral nervous systems, cardiomyopathy, and increased incidence of diabetes mellitus. The molecular consequences of the GAA triplet expansion is transcription silencing and reduced expression of the encoded mitochondrial protein, frataxin. The precise cellular role of frataxin is not known; however, it is clear now that several mitochondrial functions are not performed correctly in patient cells. The affected functions include respiration, iron—sulfur cluster assembly, iron homeostasis, and maintenance of the redox status. This review highlights the molecular mechanisms that underlie the disease phenotypes and the different hypothesis about the function of frataxin.
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This article focuses on the principal characteristics and features of the Bronze Age of the steppes, deserts, mountain foothills, and oases of Central Asia. The article examines how approaches to the social history and economy have changed from one of macro-studies of regional assemblages toward more targeted investigations of the dynamic and variable nature of this period. Finally, an overview of pottery, metal, and textile assemblages and analyses is used to form a discussion on craft production practices, consumption, and regional exchange across Central Asia.