CSPP-L and EB3 localize to centriolar satellites and are required for satellite-dependent recruitment of ciliopathy proteins to the centrosome

Objective Centrosome/Spindle Pole associated Protein 1 (CSPP1, JBTS21) mutations cause Joubert syndrome(JBTS) and JBTS-related ciliopathies. The large protein isoformCSPP-L is a ciliary protein required for ciliogenesisand stabilization of the ciliopathyprotein RPGRIP1L(NPHP8/JBTS7/MKS5/FTM) at the ciliary transition zone(TZ). However, RPGRIP1Lis dispensable for ciliogenesisand the mechanism by which CSPP-L promotes ciliogenesisis unclear.