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JHC exPRESS: First Published August 8, 2005. doi:10.1369/jhc.5A6671.2005
Copyright © Histochemical Society, Inc.

A more recent version of this article appeared on January 1, 2006.
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Articles

Tight Junction Proteins and Perineurial Cells in Neurofibromas

Kati P. Pummi 1, Heikki J. Aho 1, Matti K. Laato 1, Juha T.K. Peltonen 1 and Sirkku A. Peltonen 1*

1 Departments of Medical Biochemistry and Molecular Biology (KPP,MKL), Dermatology (KPP,SAP), Pathology (HJA), Surgery (MKL), and Anatomy (JTK), University of Turku, Turku, Finland, and Departments of Anatomy and Cell Biology (JTK), and Dermatology (JTK), University of Oulu, Oulu, Finland

* To whom correspondence should be addressed. E-mail: sirkku.peltonen{at}tyks.fi.

Submitted on February 28, 2005
Accepted on 19 July 2005


  Abstract
Cutaneous neurofibromas consist of axonal processes, Schwann cells, fibroblasts, perineurial cells, mast cells and abundant extracellular matrix. The distribution and role of perineurial cells in neurofibromas has been uncertain, partly because there has not been a specific immunohistochemical marker for perineurial cells. In this study, tight junctions of 16 neurofibromas from 12 patients with neurofibromatosis type 1 (NF1) were analyzed using electron microscopy, immunohistochemistry and western transfer analysis. Cell-cell contacts with typical ultrastructural morphology of tight junctions were seen between adjacent perineurial cells surrounding the small nerves and between contacting perineurial cell processes embedded in tumor stroma. Immunohistochemistry showed expression of claudin-1, claudin-3 and ZO-1 in the intercellular junctions of a subpopulation of tumor cells. Occludin was present mainly in perineurium and claudin-5 localized to the blood vessels. Double immunolabelings were used to identify the cell types expressing claudin-1. The results showed that claudin-1 positive cells were also positive for type IV collagen and EMA, but not for S-100 protein. This labeling pattern is consistent with perineurial cell phenotype. Using claudin-1 as a marker, our results showed that clusters of perineurial cells are distributed around the rudimentary nerves within cutaneous neurofibromas and in the periphery of some neurofibromas.

Key Words: claudin-1, EMA, neurofibroma, neurofibromatosis, perineurial cell, Schwann cell, S-100, tight junction, ZO-1


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