This Article  • Full text  • PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on ISI (8)  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Topic Collections  • Neuromuscular diseases  • Neuropathology  • Immunologic Disorders  • Alert me on articles by topic  Social Bookmarking   What's this?

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.

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati     What's this?