Exaggerated Cap-Dependent Translation as a Mechanism for Corticostriatal Dysfunction in Fragile X Syndrome Model Mice

Abstract

Our laboratories are committed to understanding the detailed molecular abnormalities associated with developmental disabilities and how these result in synaptic dysfunction and aberrant behavior. The overall hypothesis is that repetitive and perseverative behaviors exhibited by FXS patients that can be recapitulated in the FXS model mice are caused by affected cortico-striatal synapses. Our specific tasks are centered on a proteomic study of FXS striatal synapses by using a transgenic mouse model that allows to capture native post-synaptic densities. So far, we have generated the necessary number of animals, purified the synaptic complexes from different brain areas at two developmental stages and finally set up quantitative western blotting for the NMDA receptor. These experiments now enable us to identify quantitative and qualitative changes in the the protein composition of the post-synaptic densities, which will complement the electrophysiological and behavioral studies performed by the coordinator.

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Document Details

Document Type
Technical Report
Publication Date
Oct 01, 2016
Accession Number
AD1044349

Entities

People

  • Claudia Bagni
  • Eric Klann

Tags

DTIC Thesaurus Topics

  • Abnormalities
  • Biomedical Research
  • Biotechnology
  • Brain
  • Buildings And Structures
  • Diseases And Disorders
  • Dysfunction
  • Fragile-X Syndrome
  • Intellectual Disability
  • Mass Spectrometry
  • Molecular Weight
  • Nervous System Diseases
  • Neurodevelopmental Disorders
  • Neurons
  • Proteins
  • Synapses
  • Translations

Fields of Study

  • Biology

Readers

  • Immunology and Pathology
  • Marine Ecological Systems Migration
  • Neuroscience

Technology Areas

  • Biotechnology