The transition zone protein Rpgrip1l regulates proteasomal activity at the primary cilium.
Bottom Line: Mutations in RPGRIP1L result in severe human diseases called ciliopathies.Indeed, we detected a cilia-dependent decreased proteasomal activity in the absence of Rpgrip1l.We found different proteasomal components localized to cilia and identified Psmd2, a component of the regulatory proteasomal 19S subunit, as an interaction partner for Rpgrip1l.
Affiliation: Institute for Animal Developmental and Molecular Biology, Heinrich-Heine University Düsseldorf, 40225 Düsseldorf, Germany Christoph.Gerhardt@hhu.de.Show MeSH
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Mentions: It has been reported that inhibition of the proteasome leads to an accumulation of proteasomal subunits at the centrosome (Fabunmi et al., 2000). So the question arises whether proteasomal components accumulate in a significantly higher amount at the ciliary base of Rpgrip1l−/− MEFs. All analyzed proteasomal components, Psmd2, Psmd3, Psmd4 and Psma5, were significantly increased at the ciliary base in Rpgrip1l−/− MEFs (Fig. 6, A–D), underscoring the likelihood that Rpgrip1l deficiency affects proteasomal activity at primary cilia.
Affiliation: Institute for Animal Developmental and Molecular Biology, Heinrich-Heine University Düsseldorf, 40225 Düsseldorf, Germany Christoph.Gerhardt@hhu.de.