Title: IFT25 and IFT27 couple the BBSome to the IFT particle for maintenance of the ciliary signaling compartment
Abstract: Defects in cilia formation lead to a wide range of structural birth defects. Most proteins of the Intraflagellar transport (IFT) machinery –which is necessary for ciliogenesis- are highly conserved across ciliated species but IFT25 and IFT27 are absent from certain ciliated organisms suggesting that they may have a unique role distinct from cilia formation. To get better insight in their role in mammal development, we generated Ift25 and Ift27 null mice. Mutant mice die at birth with major developmental defects indicative of Hedgehog signaling dysfunction, but their cells are able to grow cilia. However, these cilia have defects in the signal-dependent transport of multiple Hedgehog components resulting in the accumulation of patched-1 and smoothened. Similarly smoothened accumulates in cilia on cells mutated for BBSome components or the BBS binding protein Lztfl1. Interestingly, the BBSome and Lztfl1 accumulate to high levels in Ift27 mutant cilia. Since Lztfl1 mutant cells accumulate BBSome but not IFT27 it is likely that Lztfl1 functions downstream of IFT27 to couple the BBSome to the IFT particle for coordinated removal of patched-1 and smoothened from cilia.
Publication Year: 2015
Publication Date: 2015-04-01
Language: en
Type: article
Indexed In: ['crossref']
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