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Primary Ciliary Deficits in the Dentate Gyrus of Fragile X Syndrome.
Stem Cell Reports ( IF 5.9 ) Pub Date : 2020-07-30 , DOI: 10.1016/j.stemcr.2020.07.001
Bumwhee Lee 1 , Shree Panda 1 , Hye Young Lee 1
Affiliation  

The primary cilium is the non-motile cilium present in most mammalian cell types and functions as an antenna for cells to sense signals. Ablating primary cilia in postnatal newborn neurons of the dentate gyrus (DG) results in both reduced dendritic arborization and synaptic strength, leading to hippocampal-dependent learning and memory deficits. Fragile X syndrome (FXS) is a common form of inheritance for intellectual disabilities with a high risk for autism spectrum disorders, and Fmr1 KO mice, a mouse model for FXS, demonstrate deficits in newborn neuron differentiation, dendritic morphology, and memory formation in the DG. Here, we found that the number of primary cilia in Fmr1 KO mice is reduced, specifically in the DG of the hippocampus. Moreover, this cilia loss was observed postnatally mainly in newborn neurons generated from the DG, implicating that these primary ciliary deficits may possibly contribute to the pathophysiology of FXS.



中文翻译:

脆性X综合征的齿状回的主要睫状缺陷。

初级纤毛是大多数哺乳动物细胞类型中存在的非活动性纤毛,并充当细胞感应信号的天线。齿状回(DG)产后新生神经元中原发纤毛的消融会导致树突状乔化和突触强度降低,从而导致海马依赖性学习和记忆障碍。脆弱X综合征(FXS)是智力障碍的一种常见遗传形式,具有自闭症谱系障碍的高风险,而FXS小鼠模型Fmr1 KO小鼠表现出新生儿神经元分化,树突形态和记忆形成方面的缺陷。 DG 在这里,我们发现Fmr1中的原纤毛数量KO小鼠减少,特别是在海马的DG中。此外,这种纤毛损失主要在出生后的DG产生的新生神经元中观察到,这说明这些原发性纤毛缺陷可能与FXS​​的病理生理有关。

更新日期:2020-07-30
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