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首页> 外文期刊>Journal of Cell Science >BBS4 is required for intraflagellar transport coordination and basal body number in mammalian olfactory cilia
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BBS4 is required for intraflagellar transport coordination and basal body number in mammalian olfactory cilia

机译：BBS4需要在哺乳动物嗅觉纤毛中的肠道颗粒运输协调和基础体数所必需的

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摘要

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 gamma-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.
机译：巴比二Beidl综合征（BBS）从编码一个或多个蛋白质BBS遗传突变清单。 BBS4损失影响嗅纤毛和气味检测，然而相关的细胞机制仍不清楚。在这里，我们报告说，BBS4（ - / - ）小鼠表现出更短的和更少的嗅感觉神经元（OSN）纤毛尽管保留气味受体定位。内BBS4（ - / - ）OSN纤毛，我们观察到的IFT-A / B的粒子移动异步率，表明在IFT复杂贩卖非协同。内的OSN树突旋钮，基础机构是动态的，异位表达的中心蛋白-2和γ微管蛋白新生ciliogenesis后发生的掺入。重要的是，在基体数的减少损失BBS4结果从纤毛损失分离。 BBS4的腺病毒表达恢复OSN纤毛长度和足以重新建立气味检测，但未能拯救纤毛和基体的数字。我们的研究结果得到的模型中的OSN多个BBS4函数包括可以解释在嗅觉神经元纤毛和ciliopathy表型的外显率的损失intraciliary和纤毛作用。

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