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Down Syndrome: From Understanding the Neurobiology to Therapy, Volume 197



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Author: Mara DierssenRafa de la Torre

Publisher: Elsevier

Publish Date: 10th May 2012

ISBN-13: 9780444543004

Pages: 272

Language: English

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Description

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.

Table of Contents

Therapeutic approaches in the improvement of cognitive performance in Down syndrome: past, present and future Rafael de la Torre and Mara Dierssen Genomic determinants in the phenotypic variability of Down syndrome Audrey Letourneau and Stylianos E. Antonarakis Intellectual disabilities, neuronal post-transcriptional RNA-metabolism and RNA-binding proteins: three actors for a complex scenario Barbara Bardoni, Sabiha Abekhoukh, Samantha Zongaro and Mireille Melko Aberrant epigenetic landscape in mental retardation Sanchez-Mut JV, Huertas D. and Esteller M. Pathways to cognitive deficits in Down syndrome Xiaolu Sturgeon, Thanh Le, Md. Mahiuddin Ahmed and Katheleen J. Gardiner Neurological Phenotypes for Down Syndrome across the Lifespan Ira T. Lott, MD Human and mouse model cognitive phenotypes in Down syndrome: Implications for assessment Jamie O. Edgin, Gina M. Mason, Goffredina Spanò, Andrea Fernández and Lynn Nadel Perturbation of dendritic protrusions in intellectual disability Josien Levenga and Rob Willemsen The in vivo Down syndrome genomic library in mouse Yann Herault, Arnaud Duchon, Emilie Velot, Damien Maréchal and Véronique Brault Discoveries in Down Syndrome: Moving Basic Science to Clinical Care A.M. Kleschevnikov, P.V. Belichenko, A. Salehi and C. Wu A Sonic hedgehog (Shh) response deficit in trisomic cells may be a common denominator for multiple features of Down syndrome Duane G. Currier, Renita C. Polk and Roger H. Reeves Gene therapy for Down syndromeCristina Fillat and Xavier Altafaj