Seminars in Pediatric Neurology
Volume 13, Issue 1 , Pages 21-26, March 2006

The Genetic and Molecular Pathogenesis of NF1 and NF2

  • Kaleb H. Yohay, MD

      Affiliations

    • Corresponding Author InformationAddress reprint requests to Kaleb H. Yohay, MD, Division of Child Neurology and Pediatrics, Johns Hopkins University, Jefferson 123, 600 North Wolfe Street, Baltimore, MD 21287-1000.

Division of Child Neurology and Pediatrics, Johns Hopkins University, Baltimore, MD.

Neurofibromatosis types 1 and 2 (NF1 and NF2) are autosomal dominant phakomatoses. The NF1 and NF2 genes encode for neurofibromin and merlin, respectively. These 2 functionally unrelated proteins both act as tumor suppressor genes, possibly through modulation of the RAS/RAC oncogenic pathways. Improved understanding of the mechanisms by which these tumor suppressors act may allow for medical therapies for neurofibromatosis and may offer insights for cancer therapeutics.

Keywords:  neurofibromatosis , NF1 , NF2 , merlin , neurofibromin , RAS , RAC

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PII: S1071-9091(06)00011-8

doi:10.1016/j.spen.2006.01.007

Seminars in Pediatric Neurology
Volume 13, Issue 1 , Pages 21-26, March 2006

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