The role of sulfoglucuronosyl glycosphingolipids in the pathogenesis of monoclonal IgM paraproteinemia and peripheral neuropathy

Hdl Handle:
http://hdl.handle.net/10675.2/673
Title:
The role of sulfoglucuronosyl glycosphingolipids in the pathogenesis of monoclonal IgM paraproteinemia and peripheral neuropathy
Authors:
Ariga, Toshio
Abstract:
In IgM paraproteinemia and peripheral neuropathy, IgM M-protein secretion by B cells leads to a T helper cell response, suggesting that it is antibody-mediated autoimmune disease involving carbohydrate epitopes in myelin sheaths. An immune response against sulfoglucuronosyl glycosphingolipids (SGGLs) is presumed to participate in demyelination or axonal degeneration in the peripheral nervous system (PNS). SGGLs contain a 3-sulfoglucuronic acid residue that interacts with anti-myelin-associated glycoprotein (MAG) and the monoclonal antibody anti-HNK-1. Immunization of animals with sulfoglucuronosyl paragloboside (SGPG) induced anti-SGPG antibodies and sensory neuropathy, which closely resembles the human disease. These animal models might help to understand the disease mechanism and lead to more specific therapeutic strategies. In an in vitro study, destruction or malfunction of the blood-nerve barrier (BNB) was found, resulting in the leakage of circulating antibodies into the PNS parenchyma, which may be considered as the initial key step for development of disease.
Citation:
Proc Jpn Acad Ser B Phys Biol Sci. 2011 Jul 25; 87(7):386-404
Issue Date:
25-Jul-2011
URI:
http://hdl.handle.net/10675.2/673
DOI:
10.2183/pjab.87.386
PubMed ID:
21785257
PubMed Central ID:
PMC3171285
Type:
Article
ISSN:
1349-2896
Appears in Collections:
Institute of Molecular Medicine and Genetics: Faculty Research and Presentations

Full metadata record

DC FieldValue Language
dc.contributor.authorAriga, Toshioen_US
dc.date.accessioned2012-10-26T16:29:29Z-
dc.date.available2012-10-26T16:29:29Z-
dc.date.issued2011-07-25en_US
dc.identifier.citationProc Jpn Acad Ser B Phys Biol Sci. 2011 Jul 25; 87(7):386-404en_US
dc.identifier.issn1349-2896en_US
dc.identifier.pmid21785257en_US
dc.identifier.doi10.2183/pjab.87.386en_US
dc.identifier.urihttp://hdl.handle.net/10675.2/673-
dc.description.abstractIn IgM paraproteinemia and peripheral neuropathy, IgM M-protein secretion by B cells leads to a T helper cell response, suggesting that it is antibody-mediated autoimmune disease involving carbohydrate epitopes in myelin sheaths. An immune response against sulfoglucuronosyl glycosphingolipids (SGGLs) is presumed to participate in demyelination or axonal degeneration in the peripheral nervous system (PNS). SGGLs contain a 3-sulfoglucuronic acid residue that interacts with anti-myelin-associated glycoprotein (MAG) and the monoclonal antibody anti-HNK-1. Immunization of animals with sulfoglucuronosyl paragloboside (SGPG) induced anti-SGPG antibodies and sensory neuropathy, which closely resembles the human disease. These animal models might help to understand the disease mechanism and lead to more specific therapeutic strategies. In an in vitro study, destruction or malfunction of the blood-nerve barrier (BNB) was found, resulting in the leakage of circulating antibodies into the PNS parenchyma, which may be considered as the initial key step for development of disease.en_US
dc.rights© 2011 The Japan Academyen_US
dc.subjectReviewen_US
dc.subject.meshAnimalsen_US
dc.subject.meshAntibodies, Monoclonalen_US
dc.subject.meshGlycosphingolipidsen_US
dc.subject.meshHumansen_US
dc.subject.meshImmunoglobulin Men_US
dc.subject.meshParaproteinemiasen_US
dc.subject.meshPeripheral Nervous Systemen_US
dc.subject.meshPeripheral Nervous System Diseasesen_US
dc.titleThe role of sulfoglucuronosyl glycosphingolipids in the pathogenesis of monoclonal IgM paraproteinemia and peripheral neuropathyen_US
dc.typeArticleen_US
dc.identifier.pmcidPMC3171285en_US
dc.contributor.corporatenameInstitute of Molecular Medicine and Genetics-

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