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.

Includes supplementary material: sn.pub/extras

Zusammenfassung
Presents an account of how genetic abnormalities, neurobiology, and neuropsychology work together to manifest cognitive-behavioral dysfunction. This work reviews the status of research in autosomal disorders that produce cognitive-behavioral dysfunction and syndromal and nonsyndromal disorders that produce mental retardation.

Inhalt
I. Introduction and Overview.- 1 The Genetics and Genomics of Neurobehavioral Disorders: Historical Introduction and Overview.- 2 Neuroanatomical Considerations Specific to the Study of Neurogenetics.- 3 Modeling Cognitive Disorders: From Genes to Therapies.- 4 What Can the Study of Behavioral Phenotypes Teach Us About the Pathway from Genes to Behavior?.- II. Autosomal Disorders and Neurobehavioral Dysfunction.- 5 The Central Nervous System in Neurofibromatosis Type 1.- 6 PraderWilli and Angelman Syndromes: Cognitive and Behavioral Phenotypes.- 7 Tuberous Sclerosis.- 8 Behavioral Phenotype in Velo-Cardio-Facial Syndrome.- 9 WilliamsBeuren Syndrome.- 10 Behavioral Phenotype in Myotonic Dystrophy (Steinert's Disease).- III. X-Linked Nonsyndromal Disorders and Neurobehavioral Dysfunction.- 11 Genetics of X-Linked Mental Retardation.- 12 Nonsyndromal Mental Retardation Associated with the FRAXE Fragile Site and the FMR2 Gene.- IV. X-Linked Syndromal Disorders and Neurobehavioral Dysfunction.- 13 ATR-X Syndrome.- 14 The Fragile X Syndrome and the Fragile X Mutation.- 15 Rett Syndrome: ClinicalMolecular Correlates.