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Downregulation traditionally refers to a reduced number of functional receptors present in cells, usually detected by reduced B max in radioligand-binding assays of tissue extracts, which occurs as a result of enhanced degradation and/or reduced biosynthesis of receptors (Tsao and von Zastrow, 2000)

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Nevertheless, from the therapeutic point of view, it is interesting to explore whether new iron chelators showing protective properties on cellular function could improve the mitochondrial dysfunction in FRDA

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