FMRP regulates the subcellular distribution of cortical dendritic spine density in a non-cell-autonomous manner.

ABSTRACT: Fragile X syndrome (FXS) is the most common form of intellectual disability that arises from the dysfunction of a single gene-Fmr1. The main neuroanatomical correlate of FXS is elevated dendritic spine density on cortical pyramidal neurons, which has been modeled in Fmr1^-/Y mice. However, the cell-autonomous contribution of Fmr1 on cortical dendritic spine density has not been assessed. Even less is known about the role of Fmr1 in heterozygous female mosaic mice, which are a putative model for human Fmr1 full mutation carriers (i.e., are heterozygous for the full Fmr1-silencing mutation). In this neuroanatomical study, spine density in cortical pyramidal neurons of Fmr1^+/- and Fmr1^-/Y mice was studied at multiple subcellular compartments, layers, and brain

SUBMITTER: Bland KM 

PROVIDER: S-EPMC7878418 | biostudies-literature | 2021 Mar

REPOSITORIES: biostudies-literature