Childhood peripheral neuropathy with autoantibodies to myelin glycoprotein P0

The serum of a 4-year-old child with severe hypertrophic peripheral neuropathy contained high-titer polyclonal antibodies, mainly of IgG class, reacting with the 30-kd P0 glycoprotein of peripheral nerve. This was demonstrated by indirect immunofluorescence, immunoblot analysis of myelin proteins, a...

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Veröffentlicht in:Annals of neurology 1992-11, Vol.32 (5), p.700-702
Hauptverfasser: Ben Jelloun-Dellagi, S, Dellagi, K, Burger, D, Ben Younes-Chennoufi, A, Hentati, F F, Steck, A, Ben Hamida, M
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container_end_page 702
container_issue 5
container_start_page 700
container_title Annals of neurology
container_volume 32
creator Ben Jelloun-Dellagi, S
Dellagi, K
Burger, D
Ben Younes-Chennoufi, A
Hentati, F F
Steck, A
Ben Hamida, M
description The serum of a 4-year-old child with severe hypertrophic peripheral neuropathy contained high-titer polyclonal antibodies, mainly of IgG class, reacting with the 30-kd P0 glycoprotein of peripheral nerve. This was demonstrated by indirect immunofluorescence, immunoblot analysis of myelin proteins, and enzyme-linked immunosorbent assay with purified P0 glycoprotein. The antibodies did not react with myelin-associated glycoprotein, sulfated glycuronic acid-containing paragloboside (SGPG and SGLPG), or other peripheral nerve glycolipids or gangliosides. To our knowledge this is the first report of strong antibody activity specifically directed against P0. This antibody may play a causative role in the pathogenesis of the peripheral neuropathy in this patient.
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subjects Autoantibodies - analysis
Cell Adhesion Molecules, Neuronal - immunology
Child, Preschool
Demyelinating Diseases - immunology
Enzyme-Linked Immunosorbent Assay
Female
Fluorescent Antibody Technique
Humans
Immunoglobulin G - immunology
Immunoglobulin M - immunology
Myelin P0 Protein
Myelin Proteins - immunology
title Childhood peripheral neuropathy with autoantibodies to myelin glycoprotein P0
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