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Chunk #40 — 3. Overview of Monogenic Mouse Models of ASDs — 3.2 Post-Transcriptional Protein Modifiers or Regulators: Fmr1, Tsc1/2, Ube3a, and Pten — 3.2.4 Ube3a (Angelman syndrome and non-syndromic ASDs)

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Monogenic mouse models of autism spectrum disorders: Common mechanisms and missing links.
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unchanged in Ube3a2xTg barrel cortex (Smith et al., 2011). Whole-cell patch clamp recording revealed reduced frequency and amplitude of mEPSCs in the dorsomedial striatum of Ube3am−/p+ mice, but not in the dorsolateral striatum (Hayrapetyan et al., 2013). Layer 2/3 pyramidal neurons in Ube3a2xTg barrel cortex display reduced eEPSC amplitude, reduced sEPSC amplitude and frequency, reduced sIPSC amplitude, and reduced mEPSC amplitude and frequency, along with decreased release probability and synaptic glutamate concentration (Smith et al., 2011). In general, the mechanisms underlying the different impairments in synapses of different brain regions remains poorly understood.