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.

Klappentext

Zur Zeit liegt uns keine Inhaltsangabe vor.

Inhalt

Part I. Introduction and Overview The Genetics and Genomics of Neurobehavioral Disorders: Historical Introduction and Overview Gene S. Fisch Neuroanatomical Considerations Specific to the Study of Neurogenetics Albert M. Galaburda and J. Eric Schmitt Modeling Cognitive Disorders: From Genes to Therapies Rui M. Costa, Ype Elgersma, and Alcino J. Silva What Can the Study of Behavioral Phenotypes Teach Us About the Pathway from Genes to Behavior? Jonathan Flint Part II. Autosomal Disorders and Neurobehavioral Dysfunction The Central Nervous System in Neurofibromatosis Type 1 Nancy Ratner and Kathryn North Prader-Willi and Angelman Syndromes: Cognitive and Behavioral Phenotypes Elisabeth M. Dykens and Suzanne B. Cassidy Tuberous Sclerosis Julian R. Sampson and Julia C. Lewis Behavioral Phenotype in Velo-Cardio-Facial Syndrome Kieran C. Murphy Williams-Beuren Syndrome Mònica Bayés and Luis A. Pérez Jurado Behavioral Phenotype in Myotonic Dystrophy (Steinert's Disease) Jean Steyaert Part III. X-Linked Nonsyndromal Disorders and Neurobehavioral Dysfunction Genetics of X-Linked Mental Retardation Jamel Chelly and Ben C. J. Hamel Nonsyndromal Mental Retardation Associated with the FRAXE Fragile Site and the FMR2 Gene Jozef Gécz and Gene S. Fisch Part IV. X-Linked Syndromal Disorders and Neurobehavioral Dysfunction ATR-X Syndrome Takahito Wada and Richard J. Gibbons The Fragile X Syndrome and the Fragile X Mutation Mark C. Hirst and Gene S. Fisch Rett Syndrome: Clinical-Molecular Correlates Alan K. Percy, Joanna Dragich, and N. Carolyn Schanen Index