Down syndrome (DS) is the most common example of neurogenetic aneuploid disorder leading to mental retardation. In most cases, DS results from an extra copy of chromosome 21 (HSA21) producing deregulated gene expression in brain that gives raise to subnormal intellectual functioning. The topic of this volume is of broad interest for the neuroscience community, because it tackles the concept of neurogenomics, that is, how the genome as a whole contributes to a neurodevelopmental cognitive disorders, such as DS, and thus to the development, structure and function of the nervous system.

This volume of Progress in Brain Research discusses comparative genomics, gene expression atlases of the brain, network genetics, engineered mouse models and applications to human and mouse behavioral and cognitive phenotypes. It brings together scientists of diverse backgrounds, by facilitating the integration of research directed at different levels of biological organization, and by highlighting translational research and the application of the existing scientific knowledge to develop improved DS treatments and cures.

Key Features

  • Leading authors review the state-of-the-art in their field of investigation and provide their views and perspectives for future research
  • Chapters are extensively referenced to provide readers with a comprehensive list of resources on the topics covered
  • All chapters include comprehensive background information and are written in a clear form that is also accessible to the non-specialist
  • Readership

    Neuroscientists, psychologists, neurologists

    Table of Contents

    1. Therapeutic approaches in the improvement of cognitive performance in Down syndrome: past, present and future
    2. Rafael de la Torre and Mara Dierssen

    3. Genomic determinants in the phenotypic variability of Down syndrome
    4. Audrey Letourneau and Stylianos E. Antonarakis

    5. Intellectual disabilities, neuronal post-transcriptional RNA-metabolism and RNA-binding proteins: three actors for a complex scenario
    6. Barbara Bardoni, Sabiha Abekhoukh, Samantha Zongaro and Mireille Melko

    7. Aberrant epigenetic landscape in mental retardation
    8. Sanchez-Mut JV, Huertas D. and Esteller M.

    9. Pathways to cognitive deficits in Down syndrome
    10. Xiaolu Sturgeon, Thanh Le, Md. Mahiuddin Ahmed and Katheleen J. Gardiner

    11. Neurological Phenotypes for Down Syndrome across the Lifespan
    12. Ira T. Lott, MD

    13. Human and mouse model cognitive phenotypes in Down syndrome: Implications for assessment
    14. Jamie O. Edgin, Gina M. Mason, Goffredina Spanò, Andrea Fernández and Lynn Nadel

    15. Perturbation of dendritic protrusions in intellectual disability
    16. Josien Levenga and Rob Willemsen

    17. The in vivo Down syndrome genomic library in mouse
    18. Yann Herault, Arnaud Duchon, Emilie Velot, Damien Maréchal and Véronique Brault

    19. Discoveries in Down Syndrome: Moving Basic Science to Clinical Care
    20. A.M. Kleschevnikov, P.V. Belichenko, A. Salehi and C. Wu

    21. A Sonic hedgehog (Shh) response deficit in trisomic cells may be a common denominator for multiple features of Down syndrome
    22. Duane G. Currier, Renita C. Polk and Roger H. Reeves

    23. Gene therapy for Down syndrome

      Cristina Fillat and Xavier Altafaj


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    © 2012
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