2023
DOI: 10.1016/j.celrep.2023.112901
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Cell-type-specific disruption of cortico-striatal circuitry drives repetitive patterns of behavior in fragile X syndrome model mice

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Cited by 8 publications
(3 citation statements)
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“…FXS is a genetic form of intellectual disability and ASD characterized by the genetic inactivation of Fmrp , encoding for an RNA-binding protein that represses the translation of a specific subset of mRNAs associated with eIF4E. Thus, FXS model mice exhibit increased eIF4E-dependent protein synthesis along with hyperactive mTOR signaling 83,84 . The dysregulated protein synthesis is accompanied by diminished evoked DA release detected by FSCV in acute corticostriatal slices 85 and behavioral inflexibility in a touchscreen operant task 86 .…”
Section: Discussionmentioning
confidence: 99%
“…FXS is a genetic form of intellectual disability and ASD characterized by the genetic inactivation of Fmrp , encoding for an RNA-binding protein that represses the translation of a specific subset of mRNAs associated with eIF4E. Thus, FXS model mice exhibit increased eIF4E-dependent protein synthesis along with hyperactive mTOR signaling 83,84 . The dysregulated protein synthesis is accompanied by diminished evoked DA release detected by FSCV in acute corticostriatal slices 85 and behavioral inflexibility in a touchscreen operant task 86 .…”
Section: Discussionmentioning
confidence: 99%
“…Moreover, these compounds have been observed to normalize enhanced protein synthesis and mGluR-mediated LTD in the hippocampus in a Fragile X syndrome mouse model ( Thomson et al, 2017 ). Indeed, behavior abnormalities including RRB, excessive grooming and altered LTD, are rescued in this mouse model by administration of VU0152100, a positive allosteric modulator of MR4 ( Longo et al, 2023 ).…”
Section: Cellular and Molecular Mechanisms Of Repetitive Behaviormentioning
confidence: 95%
“…Furthermore, cell-type specific alterations have been taken into account in the Fmr1 KO mouse, a model of FXS characterized by RRB, marble-burying, nestlet shredding tests and self-grooming behavior. Longo and colleagues report dysregulated de novo protein synthesis specifically in dSPNs, due to an increased de novo cap-dependent translation ( Longo et al, 2023 ). Moreover, they find increased spine density in dSPNs of the DLS in the Fmr1KO mouse while no changes are detected in iSPNs.…”
Section: Cellular and Molecular Mechanisms Of Repetitive Behaviormentioning
confidence: 99%