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- W2910002973 abstract "Bardet-Beidl Syndrome (BBS) manifests from genetic mutations encoding for one or more BBS proteins. BBS4 loss impacts olfactory ciliation and odor detection yet the cellular mechanisms remain unclear. Here, we report that Bbs4−/− mice exhibit shorter and fewer olfactory sensory neuron (OSN) cilia despite retaining odorant receptor localization. Within Bbs4−/− OSN cilia, we observed asynchronous rates of IFT-A/B particle movements indicating miscoordination in IFT complex trafficking. Within the OSN dendritic knob, the basal bodies are dynamic with incorporation of ectopically expressed centrin-2 and γ-tubulin occurring after nascent ciliogenesis. Importantly, BBS4 loss results in the reduction of basal body numbers separate from cilia loss. Adenoviral expression of BBS4 restored OSN cilia lengths and was sufficient to re-establish odor detection but failed to rescue ciliary and basal body numbers. Our results yield a model for the plurality of BBS4 functions in OSNs that includes intraciliary and periciliary roles that can explain the loss of cilia and penetrance of ciliopathy phenotypes in olfactory neurons." @default.
- W2910002973 created "2019-01-25" @default.
- W2910002973 creator A5000288080 @default.
- W2910002973 creator A5014285260 @default.
- W2910002973 creator A5023904964 @default.
- W2910002973 creator A5023986637 @default.
- W2910002973 creator A5031191250 @default.
- W2910002973 creator A5048989892 @default.
- W2910002973 creator A5049389962 @default.
- W2910002973 creator A5074090267 @default.
- W2910002973 creator A5078061167 @default.
- W2910002973 creator A5081318736 @default.
- W2910002973 date "2019-01-01" @default.
- W2910002973 modified "2023-10-05" @default.
- W2910002973 title "BBS4 is required for IFT coordination and basal body number in mammalian olfactory cilia." @default.
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