Modeling cognitive dysfunction in neurofibromatosis-1
نویسندگان
چکیده
منابع مشابه
Modeling cognitive dysfunction in neurofibromatosis-1.
Cognitive dysfunction, including significant impairments in learning, behavior, and attention, is found in over 10% of children in the general population. However, in the common inherited cancer predisposition syndrome, neurofibromatosis type 1 (NF1), the prevalence of these cognitive deficits approaches 70%. As a monogenic disorder, NF1 provides a unique genetic tool to identify and dissect me...
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Neurofibromatosis type 1 (NF1) is the most common monogenic disorder in which individuals manifest CNS abnormalities. Affected individuals develop glial neoplasms (optic gliomas, malignant astrocytomas) and neuronal dysfunction (learning disabilities, attention deficits). Nf1 genetically engineered mouse models have revealed the molecular and cellular underpinnings of gliomagenesis, attention d...
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Objective Neurofibromatosis type 1 (NF1) causes neural and cutaneous disorders and reduced exercise capacity. Exercise/heat exposure increasing internal temperature must be compensated by eccrine sweat function and warmed skin vasodilation. We suspected NF1 could adversely affect eccrine sweat function and/or vascular thermoregulatory responses (VTR). Methods The eccrine sweat function and VT...
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Aim The last systematic review of research on the behavior of children with neurofibromatosis type 1 (NF1) was in 2012. Since then, several important findings have been published. Therefore, the study aim was to synthesize recent relevant work related to this issue. Method We conducted a systematic review of the literature. Relevant articles were identified using the electronic databases PubM...
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Neurofibromatosis type 1 (NF1) is a disorder caused by mutations in a single gene. These mutations result in neurological dysfunction including learning disabilities in children and cognitive impairment in adults. Individuals with NF1 often develop benign and sometimes malignant neoplasms of the brain and peripheral nerves. The underlying gene responsible for these defects is the NF1 gene, whic...
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ژورنال
عنوان ژورنال: Trends in Neurosciences
سال: 2013
ISSN: 0166-2236
DOI: 10.1016/j.tins.2012.12.002