Merlin, the NF2 Gene Product

Springer Science and Business Media LLC - Tập 19 - Trang 365-373 - 2013
Nives Pećina-Šlaus1,2
1Laboratory of Neurooncology, Croatian Institute for Brain Research, School of Medicine, University of Zagreb, Zagreb, Croatia
2Department of Biology, School of Medicine, University of Zagreb, Zagreb, Croatia

Tóm tắt

Merlin, the protein product of NF2 gene, is one of the most versatile tumor suppressors capable of integrating different mechanisms that regulate cell proliferation, motility, survival and signaling pathways underlying and governing those mechanisms. Merlin is considered a member of the band 4.1 families of cytoskeleton-associated proteins also called ERM family and acts as tumor suppressor. The main cause for transformation of Schwann cells into schwannomas is credited to the inactivation of the neurofibromin 2 (NF2) gene and the consecutive loss of its protein merlin. Recent scientific advances improved our understanding of pathogenic mechanisms involving NF2 gene. The present review brings genetic properties of NF2 gene, molecular characteristics of merlin, summarizes mutational spectra and explains merlin’s multifunctional roles regarding its involvement in neurofibromatosis associated tumorigenesis.

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