Journal
TRENDS IN NEUROSCIENCES
Volume 36, Issue 4, Pages 237-247Publisher
ELSEVIER SCIENCE LONDON
DOI: 10.1016/j.tins.2012.12.002
Keywords
neurofibromin; cyclic AMP; dopamine; NF1; learning; attention deficits
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Funding
- Department of Defense [W81XWH-10-1-0884]
- National Cancer Institute [U01-CA141549]
- Diversity Supplement from the National Cancer Institute [U01-CA141549]
- NATIONAL CANCER INSTITUTE [U01CA141549] Funding Source: NIH RePORTER
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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 mechanistically the molecular and cellular bases underlying cognitive dysfunction. In this review, we discuss Nf1 fly and mouse systems that mimic many of the cognitive abnormalities seen in children with NF1. Further, we describe discoveries from these models that have uncovered defects in the regulation of Ras activity, cAMP generation, and dopamine homeostasis as key mechanisms important for cognitive dysfunction in children with NF1.
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