Primary Ciliary Deficits in the Dentate Gyrus of Fragile X Syndrome

Bumwhee Lee, Shree Panda, Hye Young Lee

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish (US)
Pages (from-to)454-466
Number of pages13
JournalStem Cell Reports
Volume15
Issue number2
DOIs
StatePublished - Aug 11 2020

Keywords

  • dentate gyrus
  • fragile X syndrome
  • neurodevelopmental disorders
  • newborn neurons
  • primary cilia
  • subgranular zone

ASJC Scopus subject areas

  • Biochemistry
  • Genetics
  • Developmental Biology
  • Cell Biology

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