Delayed onset of striatal projection neuron hyperexcitability in <i>Fmr1-/y</i> mice.

ABSTRACT:

Introduction

Fragile X Syndrome (FXS), the most common genetic cause of intellectual disability and autism spectrum disorder (ASD), results from silencing of the FMR1 gene and consequent loss of Fragile X Messenger Ribonucleoprotein (FMRP). FMRP deficiency disrupts neural development, leading to behavioral and motor deficits associated with striatal dysfunction. Although structural and functional abnormalities in striatal projection neurons (SPNs) have been observed in adult Fmr1 knockout mice (Fmr1-/y), their developmental onset and contribution to early FXS pathophysiology remain unknown.

Methods

We examined the postnatal maturation of SPNs in the dorsomedial striatum (DMS) of Fmr1-/y mice, assessing glutamatergic synaptic inputs and intrinsic e

SUBMITTER: Nelson L 

PROVIDER: S-EPMC12657441 | biostudies-literature | 2025

REPOSITORIES: biostudies-literature