KIF13B establishes a CAV1-enriched microdomain at the ciliary transition zone to promote Sonic hedgehog signalling.

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Kenneth Bødtker Schou, Johanne Bay Mogensen, Stine Kjær Morthorst, Brian Skriver Nielsen, Aiste Aleliunaite, Andrea Serra-Marques, Nicoline Fürstenberg, Sophie Saunier, Albane A. Bizet, Iben Rønn Veland, Anna Akhmanova, Søren Tvorup Christensen, Lotte Bang Pedersen

Ciliary membrane composition is controlled by transition zone (TZ) proteins such as RPGRIP1, RPGRIPL and NPHP4, which are vital for balanced coordination of diverse signalling systems like the Sonic hedgehog (Shh) pathway. Activation of this pathway involves Shh-induced ciliary accumulation of Smoothened (SMO), which is disrupted by disease-causing mutations in TZ components. Here we identify kinesin-3 motor protein KIF13B as a novel member of the RPGRIP1N-C2 domain-containing protein family and show that KIF13B regulates TZ membrane composition and ciliary SMO accumulation. KIF13B is upregulated during ciliogenesis and is recruited to the ciliary base by NPHP4, which binds to two distinct sites in the KIF13B tail region, including an RPGRIP1N-C2 domain. KIF13B and NPHP4 are both essential for establishment of a CAV1 membrane microdomain at the TZ, which in turn is required for Shh-induced ciliary SMO accumulation. Thus KIF13B is a novel regulator of ciliary TZ configuration, membrane composition and Shh signalling.
Original languageEnglish
Article number14177
JournalNature Communications
Volume8
Number of pages15
ISSN2041-1723
DOIs
Publication statusPublished - 30 Jan 2017

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