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Pathogenic role of ganglioside metabolism in neurodegenerative diseases
Author(s) -
Ariga Toshio
Publication year - 2014
Publication title -
journal of neuroscience research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.72
H-Index - 160
eISSN - 1097-4547
pISSN - 0360-4012
DOI - 10.1002/jnr.23411
Subject(s) - neurodegeneration , lipid raft , amyotrophic lateral sclerosis , ganglioside , lipid metabolism , pathogenesis , biology , disease , huntingtin , huntington's disease , neuroscience , microbiology and biotechnology , immunology , medicine , signal transduction , biochemistry , pathology
Ganglioside metabolism is altered in several neurodegenerative diseases, and this may participate in several events related to the pathogenesis of these diseases. Most changes occur in specific areas of the brain and their distinct membrane microdomains or lipid rafts. Antiganglioside antibodies may be involved in dysfunction of the blood–brain barrier and disease progression in these diseases. In lipid rafts, interactions of glycosphingolipids, including ganglioside, with proteins may be responsible for the misfolding events that cause the fibril and/or aggregate processing of disease‐specific proteins, such as α‐synuclein, in Parkinson's disease, huntingtin protein in Huntington's disease, and copper‐zinc superoxide dismutase in amyotrophic lateral sclerosis. Targeting ganglioside metabolism may represent an underexploited opportunity to design novel therapeutic strategies for neurodegeneration in these diseases. © 2014 Wiley Periodicals, Inc.

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