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Se delivery to neurons by SELENOP is accomplished via its receptor, low-density lipoprotein receptor-related protein 8 (LRP8, also known as ApoER2, Figure 1) ( choroid plexus epithelial cells (Olson et al., 2007, 2008

Of notice, loss-of-function mutations of PNKP in humans are also a cause of ataxia [106, 107], reinforcing the link between the ATXN3-related networks and cerebellar dysfunction