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Immunolocalization and Activation of Transcription Factor Nuclear Factor  B in Dysimmune Neuropathies and Familial Amyloidotic Polyneuropathy
Anna Mazzeo, MD;
Mohammed Aguennouz, PhD;
Corrado Messina, MD;
Giuseppe Vita, MD
Arch Neurol. 2004;61:1097-1102.
Background Recently, immunoreactivity of transcription factor nuclear factor  B (NF-B) was found in peripheral nerves from patients with Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy (CIDP), and familial amyloidotic polyneuropathy (FAP), suggesting a role in their pathogenesis.
Objective To investigate expression and activation of NF-B in nerve biopsy specimens from patients with peripheral neuropathies of different origins.
Patients Nerve biopsies from 17 patients (5 with CIDP, 3 with vasculitis, 4 with Charcot-Marie-Tooth disease, and 5 with FAP) and 3 normal sural nerves were studied by immunocytochemistry and Western blot of nuclear extracts for the activated form of NF-B. Nuclear factor B DNA-binding activity was studied by electrophoretic mobility shift assay.
Results Immunobinding for the activated form p65 of NF-B was found in 2% to 5% of endoneurial vessel walls, in the external myelin of 5% to 10% of fibers, and in a few axons in CIDP specimens. It was also found in 5% to 15% of epineurial and endoneurial vessels in vasculitis specimens and at the level of amyloid deposits in FAP nerves. Nuclear factor B immunoreactivity was not correlated to type of inflammatory cells, but it often corresponded to the deposition of the terminal complement complex C5b9. Western blot analysis of nuclear extracts showed a single band corresponding to 65 kDa in all affected nerves. Nuclear factor B DNA-binding activity was revealed by electrophoretic mobility shift assay in specimens from patients with CIDP, vasculitis, and FAP.
Conclusion Our novel findings suggest a crucial role of NF-B in inflammatory neuropathies and FAP.
From the Department of Neuroscience, Psychiatry, and Anesthesiology, University of Messina, Messina, Italy.
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