MKS and NPHP modules cooperate to establish basal body/transition zone membrane associations and ciliary gate function during ciliogenesis.
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MKS-3 localizes to the ciliary transition zone in C. elegans sensory neurons
"Using fluorescently tagged proteins, we detect MKS/MKSR/NPHP proteins in a region corresponding to the TZ (adjacent to where IFT proteins concentrate at the TFs/BB). This is evident for MKS-1, MKS-1 related-1 (MKSR-1)/B9D1, MKS-1 related-2 (MKSR-2)/B9D2, MKS-3/meckelin, NPHP-1, and NPHP-4
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MKS-3 is a component of the MKS module that functions with the NPHP module in ciliogenesis
"We find that MKS-5/RPGRIP1L interacts with two distinct TZ functional modules, MKS/MKSR and NPHP, consisting of MKS-1/MKSR-1/MKSR-2/MKS-3/MKS-6 and NPHP-1/NPHP-4 proteins, respectively.
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MKS-3 participates in establishing basal body/TZ membrane attachments during early ciliogenesis
"Functional interactions between different MKS module components and the NPHP module are essential for an IFT-independent early stage of ciliogenesis, namely docking/anchoring of the BB/TZ to the membrane.
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MKS-3 is required for ciliary gate function that restricts non-ciliary proteins from entering cilia
"Together, our data showing abnormal accumulations of MKS-3, RPI-2, and TRAM-1a in TZ mutant cilia indicate that, in addition to their role in early ciliogenesis, most if not all TZ proteins normally function to maintain a boundary at the cilium base, establishing the TZ as a bona fide ciliary gate.
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mks-3;nphp-4 double mutants show severe ciliogenesis defects including missing Y-links and TZ detachment
"Although similar ciliary defects were observed in the latter mutants (Fig. 7; Fig. S4, Fig. S5), some of their TZ regions are in normal proximity to the ciliary membrane but often lack connecting Y-links
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