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EXPRESSION OF TENASCIN‐C IN THE SURAL NERVE OF PATIENTS WITH INHERITED DEMYELINATING NEUROPATHIES
Author(s) -
Massa R.,
Palumbo C.,
Bernardi G.,
Modesti A.
Publication year - 2000
Publication title -
journal of the peripheral nervous system
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1
H-Index - 67
eISSN - 1529-8027
pISSN - 1085-9489
DOI - 10.1046/j.1529-8027.2000.00513-38.x
Subject(s) - remyelination , node of ranvier , schwann cell , sural nerve , pathology , myelin , biology , axon , peripheral nervous system , chronic inflammatory demyelinating polyneuropathy , neuroscience , anatomy , medicine , central nervous system , immunology , antibody
Tenascin‐C (TN‐C) is an extracellular matrix glycoprotein synthesized by glial cells that is involved in neuron–glia interactions. During development of peripheral nerve (PN), TN‐C is abundantly expressed by myelinating Schwann cells, while in the adult its synthesis is down‐regulated and its localization restricted to the node of Ranvier in myelinated nerve fibers. Interestingly, TN‐C expression is up‐regulated in the PN of some animal models of inherited human demyelinating neuropathies. Moreover, in TN‐C‐deficient mice, important morphological alterations of PN have been demonstrated. We have therefore studied the expression of TN‐C in sural nerve biopsies of patients with inherited demyelinating neuropathies such as CMT1 and HNPP, using immunoperoxidase and immunofluorescence methods. At variance with control nerves, where TN‐C immunoreactivity was localized only in the perineurium and at the node of Ranvier, in all pathological nerves TN‐C was present also in a variable number of Schwann cells and myelin sheaths, sometimes marking whole myelin internodes. These data suggest that TN‐C expression by Schwann cells in human pathologic nerves may be a marker of active demyelination and/or remyelination. These events may be modulated by TN‐C by mediating adhesive interactions between axons and Schwann cells, in a way similar to that observed in the developmental stage.