Expression of NF2-encoded Merlin and Related ERM Family Proteins in the Human Central Nervous System

Germline mutations of the neurofibromatosis 2 (NF2) gene are associated with an increased incidence of gliomas and glial hamartomas, suggesting a role for the NF2-encoded protein, merlin, in glial growth control. Using monoclonal and polyclonal anti-merlin antibodies for Western blotting and immunoh...

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Bibliographic Details
Published in:Journal of neuropathology and experimental neurology 1997-06, Vol.56 (6), p.735-742
Main Authors: Stemmer-Rachamimov, Anat O, Gonzalez-Agosti, Charo, Xu, Lin, Burwick, Jennifer A, Beauchamp, Roberta, Pinney, Denise, Louis, David N, Ramesh, Vuaya
Format: Article
Language:English
Subjects:
Adult
Astrocytes - chemistry
Biological and medical sciences
Blood Proteins - analysis
Blotting, Western
Central Nervous System - chemistry
Cytoskeletal Proteins
Genes, Neurofibromatosis 2
Gliosis - metabolism
Hamartoma - metabolism
Humans
Immunohistochemistry
Medical sciences
Membrane Proteins - analysis
Microfilament Proteins
Middle Aged
Neurofibromin 2
Neurology
Phosphoproteins - analysis
Proteins - analysis
Tumors of the nervous system. Phacomatoses
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Summary:Germline mutations of the neurofibromatosis 2 (NF2) gene are associated with an increased incidence of gliomas and glial hamartomas, suggesting a role for the NF2-encoded protein, merlin, in glial growth control. Using monoclonal and polyclonal anti-merlin antibodies for Western blotting and immunohistochemistry, we evaluated the cellular pattern of merlin expression in the normal human central nervous system (CNS), reactive gliosis, and NF2-associated glial hamartomas. In the normal CNS. merlin is widely expressed in coarse cytoplasmic granules in both glia and neurons. with less pronounced expression in other cells. Merlin is also expressed in reactive astrocytes and in the astrocytes of NF2-associated glial hamartomas. In reactive astrocytes, however, merlin is also present at the cell membrane and in cellular processes, suggesting redistribution of the protein in activated cells. Merlin is structurally related to ezrin, radixin and moesin, which are also expressed in the CNS, as demonstrated by Western blotting. The pattern of merlin expression, however, is distinct from that of ezrin, which has been previously described, and that of moesin, in which immunohistochemistry with an anti-moesin antibody showed expression in endothelial cells, glia and neurons in a membranous or diffuse cytoplasmic pattern. These findings imply that merlin has widespread and specific functions in the human central nervous system.
ISSN:0022-3069
1554-6578
DOI:10.1097/00005072-199706000-00011