Neuronal survival critically depends on the integrity and functionality of mitochondria. A hierarchical system of control mechanisms protects mitochondria against stress, monitors mitochondrial damage and ensures the selective removal of dysfunctional mitochondrial proteins or organelles. Moreover well-known transcriptional cascades control mitochondrial biogenesis under different metabolic conditions. In contrast, little is known about post-transcriptional mechanisms of regulation of mitochondrial function. Recently, we have identified a novel RNA-binding protein, CLUH, which specifically bind mRNAs encoding mitochondrial proteins. We aim to understand the molecular mechanisms underlying CLUH function and the physiological and pathological conditions in which CLUH is implicated.