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Annu Rev Neurosci ; 33: 221-43, 2010.
Artigo em Inglês | MEDLINE | ID: mdl-20345245

RESUMO

Neurofibromatosis Type I (NF1) is a single-gene disorder characterized by a high incidence of complex cognitive symptoms, including learning disabilities, attention deficit disorder, executive function deficits, and motor coordination problems. Because the underlying genetic cause of this disorder is known, study of NF1 from a molecular, cellular, and systems perspective has provided mechanistic insights into the etiology of higher-order cognitive symptoms associated with the disease. In particular, studies of animal models of NF1 indicated that disruption of Ras regulation of inhibitory networks is critical to the etiology of cognitive deficits associated with NF1. Animal models of Nf1 identified mechanisms and pathways that are required for cognition, and represent an important complement to the complex neuropsychological literature on learning disabilities associated with this condition. Here, we review findings from NF1 animal models and human populations affected by NF1, highlighting areas of potential translation and discussing the implications and limitations of generalizing findings from this single-gene disease to idiopathic learning disabilities.


Assuntos
Deficiências da Aprendizagem/genética , Deficiências da Aprendizagem/fisiopatologia , Neurofibromatose 1/genética , Neurofibromatose 1/fisiopatologia , Proteínas ras/genética , Animais , Modelos Animais de Doenças , Humanos , Neurofibromatose 1/complicações , Proteínas ras/metabolismo
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