Skip Navigation

This Article
Right arrow FREE Full Text (PDF) Freely available
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrow Search for citing articles in:
ISI Web of Science (33)
Right arrowRequest Permissions
Right arrow Disclaimer
Google Scholar
Right arrow Articles by Gabriel, J.-M.
Right arrow Articles by Steck, A. J.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Gabriel, J.-M.
Right arrow Articles by Steck, A. J.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

Brain, Vol. 119, No. 3, 775-787, 1996
© 1996 Guarantors of Brain

research-article

Selective loss of myelin-associated glycoprotein from myelin correlates with anti-MAG antibody titre in demyelinating paraproteinaemic polyneuropathy

J.-M. Gabriel1, B. Erne1, G. C. Miescher1, S. L. Miller3, A. Vital4, C. Vital4 and A. J. Steck2,

1Departments of Research and Clinical Neurology, University Hospital Basel Switzerland 2Departments of Clinical Neurology, University Hospital Basel Switzerland 3Division of Neurology Research, Children's Hospital Philadelphia, USA 4Service d'Anatomie Pathologique, Hopital Pellegrin Bordeaux, France

Correspondence to: Correspondence to: A. J. Steck, Neurology Clinic, University Hospital Basel, Petersgraben 4, CH-4031 Basel, Switzerland

The IgM monoclonal autoantibodies of patients with demyelinating paraproteinaemic polyneuropathy recognize a carbohydrate structure present on both myelin-associated glycoprotein (MAG) and protein zero (Po). These autoantibodies are sufficient to cause the disease but the mechanism of demyelination remains unclear. We have analysed nerve biopsies from eight patients with polyneuropathy and anti-MAG antibodies by quantitative immunohistochemistry and find a concordant pattern of reduced expression of myelin markers with the loss of myelinated fibres. We report here novel features of this disease, in particular a selective lack of detectable MAG in a large proportion of myelinated fibres containing Po, myelin basic protein (MBP) and periaxin. There is also an inverse correlation of the distribution of MAG in peripheral never myelin with the serum anti-MAG antibody titres but no correlation of these titres with the loss of myelinated fibres. Double immunofluorescence staining of paraproteinaemic polyneuropathy (PPN) nerves shows anti-MAG IgM deposited on the periphery of myelinated fibres associated with or lacking MAG staining. These data suggest that the binding of anti-MAG antibodies to MAG and/or other myelin component(s) results in MAG downregulation and may have an essential role in the molecular mechanisms leading to demyelination and partial regeneration in this disease.

myelin-associated glycoprotein; anti-MAG autoantibodies; paraproteinaemic polyneuropathy; enzyme immunohistochemistry; quantitative image analysis

Received November 10, 1995. Revised January 24, 1996. Accepted February 6, 1996.


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?


This article has been cited by other articles:


Home page
 NeurologyHome page
M. Eurelings, K.G.M. Moons, N.C. Notermans, L.D. Sasker, A.E.J. De Jager, A.R. Wintzen, J.H.J. Wokke, and L.H. Van den Berg
Neuropathy and IgM M-proteins: Prognostic value of antibodies to MAG, SGPG, and sulfatide
Neurology, January 23, 2001; 56(2): 228 - 233.
[Abstract] [Full Text] [PDF]

Home page
 NeuroscientistHome page
R. D. Fields
Cell Adhesion Molecules: Implications for Neurological Disease
Neuroscientist, January 1, 1998; 4(1): 4 - 8.
[PDF]


Disclaimer: Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department.