Modeling Fragile X Syndrome

1. Front Matter

   Pages i-xi

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2. Ex Vivo Models

   1. Front Matter

      Pages 13-13

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   2. Introduction: Reminiscing on Models and Modeling

      • Robert B. Denman

      Pages 1-12

   3. Probing Astrocyte Function in Fragile X Syndrome

      • Shelley Jacobs, Connie Cheng, Laurie C. Doering

      Pages 15-31

   4. Neural Stem Cells

      • Maija Castrén

      Pages 33-40

   5. Fragile X Mental Retardation Protein (FMRP) and the Spinal Sensory System

      • Theodore J. Price, Ohannes K. Melemedjian

      Pages 41-59

   6. The Role of the Postsynaptic Density in the Pathology of the Fragile X Syndrome

      • Stefan Kindler, Hans-Jürgen Kreienkamp

      Pages 61-80

3. Non-Mouse Eukaryote Models

   1. Front Matter

      Pages 81-81

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   2. Behavior in a Drosophila Model of Fragile X

      • Sean M. McBride, Aaron J. Bell, Thomas A. Jongens

      Pages 83-117

   3. Molecular and Genetic Analysis of the Drosophila Model of Fragile X Syndrome

      • Charles R. Tessier, Kendal Broadie

      Pages 119-156

   4. Fragile X Mental Retardation Protein and Stem Cells

      • Abrar Qurashi, Xuekun Li, Peng Jin

      Pages 157-164

   5. Manipulating the Fragile X Mental Retardation Proteins in the Frog

      • Marc-Etienne Huot, Nicolas Bisson, Thomas Moss, Edouard W. Khandjian

      Pages 165-179

   6. Exploring the Zebra Finch Taeniopygia guttata as a Novel Animal Model for the Speech–Language Deficit of Fragile X Syndrome

      • Claudia Winograd, Stephanie Ceman

      Pages 181-197

4. Novel Mouse Models

   1. Front Matter

      Pages 199-199

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   2. Neuroendocrine Alterations in the Fragile X Mouse

      • Abdeslem El Idrissi, Xin Yan, William L’Amoreaux, W. Ted Brown, Carl Dobkin

      Pages 201-221

   3. Taking STEPs Forward to Understand Fragile X Syndrome

      • Susan M. Goebel-Goody, Paul J. Lombroso

      Pages 223-241

   4. Fmr-1 as an Offspring Genetic and a Maternal Environmental Factor in Neurodevelopmental Disease

      • Bojana Zupan, Miklos Toth

      Pages 243-253

   5. Mouse Models of the Fragile X Premutation and the Fragile X Associated Tremor/Ataxia Syndrome

      • Michael R. Hunsaker, Gloria Arque, Robert F. Berman, Rob Willemsen, Renate K. Hukema

      Pages 255-269

5. The Human Model

   1. Front Matter

      Pages 271-271

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   2. Clinical Aspects of the Fragile X Syndrome

      • W. Ted Brown

      Pages 273-279

Introduction.-Probing Astrocyte Function in Fragile X Syndrome.- Neural Stem Cells.- Fragile X Mental Retardation Protein (FMRP) and the Spinal Sensory System.– The Role of the Postsynaptic Density in the Pathology of the Fragile X Syndrome.- Behavior in a Drosophila model of Fragile X.- Molecular and Genetic Analysis of the Drosophila Model of Fragile X Syndrome.- Fragile X Mental Retardation Protein and Stem Cells.- Manipulating the Fragile X Mental Retardation Proteins in the Frog.- Exploring the Zebra finch Taeniopygia gutta as a Novel Animal Model for the Speech-language Deficit of Fragile X Syndrome.- Neuroendocrine Alterations in the Fragile X Mouse.- Taking STEPs forward to understanding Fragile X Syndrome.- Fmr-1 as an Offspring Genetic and a Maternal Environmental Factor in Neurodevelopmental Disease.- Mouse Models of the Fragile X Premutation and the Fragile X Associated Tremor/Ataxia Syndrome.- Clinical Aspects of the Fragile X Syndrome.- Fragile X Syndrome: A Psychiatric Perspective.- Fragile X Syndrome and Targeted Treatment Trials.- The Fragile X-associate Tremor Ataxia Syndrome.- Vignettes: Models in Absentia.

• Epigenetics
• FMR1
• FMR1 Gene
• FXS
• Fragile X Syndrome
• Genetic disorder
• Inherited disease
• Stem cells
• gene therapy

• New York State Institute for Basic Resea, Staten Island, USA

  Robert B. Denman

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