Genetics and genomics of neurobehavioral disorders / edited by Gene S. Fisch.

Format:

Book

Contributor:

Fisch, Gene S.

Series:

Contemporary clinical neuroscience

Language:

English

Subjects (All):

Neurobehavioral disorders--Genetic aspects.

Neurobehavioral disorders.

Neurobehavioral Manifestations--genetics.

Medical Subjects:

Neurobehavioral Manifestations--genetics.

Physical Description:

x, 428 pages : illustrations ; 24 cm.

Place of Publication:

Totowa, N.J. : Humana Press, [2003]

Summary:

In recent years, significant gains have been made in the cloning and identification of numerous genes that produce neurobehavioral disorders, as well as in the ability of neuropsychological tests to accurately assess and evaluate cognitive deficits and behavioral dysfunction. In Genetics and Genomics of Neurobehavioral Disorders, a panel of leading researchers draws on this new knowledge to provide a clear and comprehensive account of how genetic abnormalities, neurobiology, and neuropsychology work together to manifest cognitive-behavioral dysfunction. The authors review the current status of research in autosomal disorders that produce such dysfunctions, examining both microdeletion disorders (Prader-Willi syndrome/Angelman syndrome, deletion 22q11, and Williams syndrome) and syndromes arising from microdeletions and sequence variants, among them neurofibromatosis (NF1 and NF2), tuberous sclerosis (TSC1 and TSC2), and myotonic dystrophy. X-linked disorders producing mental retardation in syndromal disorders (ATRX, Rett, and fragile X) and nonsyndromal disorders (FRAXE and MRX) are also discussed. An insightful introduction surveys the history of neurobehavioral disorders, the establishment of relationships between genetic and cognitive impairment, the relationship between neurobiology and behavior, recent advances in neurogenomics, and the use of animal models for complex human disorders and cognitive impairment. Comprehensive and up-to-date, Genetics and Genomics of Neurobehavioral Disorders integrates the molecular, genomic, neuropsychological, and neurobehavioral factors that produce learning disabilities and mental retardation into a coherent framework for the understanding and assessment of neurobehavioral disorders.

Contents:

1 The Genetics and Genomics of Neurobehavioral Disorders: Historical Introduction and Overview / Gene S. Fisch 3

2 Neuroanatomical Considerations Specific to the Study of Neurogenetics / Albert M. Galaburda, J. Eric Schmitt 21

3 Modeling Cognitive Disorders: From Genes to Therapies / Rui M. Costa, Ype Elgersma, Alcino J. Silva 39

4 What Can the Study of Behavioral Phenotypes Teach Us About the Pathway from Genes to Behavior? / Jonathan Flint 69

Part II. Autosomal Disorders and Neurobehavioral Dysfunction

5 The Central Nervous System in Neurofibromatosis Type 1 / Nancy Ratner, Kathryn North 97

6 Prader-Willi and Angelman Syndromes: Cognitive and Behavioral Phenotypes / Elisabeth M. Dykens, Suzanne B. Cassidy 133

7 Tuberous Sclerosis / Julian R. Sampson, Julia C. Lewis 159

8 Behavioral Phenotype in Velo-Cardio-Facial Syndrome / Kieran C. Murphy 195

9 Williams-Beuren Syndrome / Monica Bayes, Luis A. Perez Jurado 209

10 Behavioral Phenotype in Myotonic Dystrophy (Steinert's Disease) / Jean Steyaert 237

Part III. X-Linked Nonsyndromal Disorders and Neurobehavioral Dysfunction

11 Genetics of X-Linked Mental Retardation / Jamel Chelly, Ben C. J. Hamel 263

12 Nonsyndromal Mental Retardation Associated with the FRAXE Fragile Site and the FMR2 Gene / Jozef Gecz, Gene S. Fisch 289

Part IV. X-Linked Syndromal Disorders and Neurobehavioral Dysfunction

13 ATR-X Syndrome / Takahito Wada, Richard J. Gibbons 309

14 The Fragile X Syndrome and the Fragile X Mutation / Mark C. Hirst, Gene S. Fisch 335

15 Rett Syndrome: Clinical-Molecular Correlates / Alan K. Percy, Joanna Dragich, N. Carolyn Schanen 391.

Notes:

Includes bibliographical references and index.

ISBN:

158829045X

OCLC:

49959257