title: Pathogenic role of ganglioside metabolism in neurodegenerative diseases.
abstract: 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.
authors.affiliation: Institute of Molecular Medicine and Genetics, Institute of Neuroscience, Medical College of Georgia, Georgia Regents University, Augusta, Georgia.
authors.forename: Toshio
authors.initials: T
authors.lastname: Ariga
mesh_terms: D000818:Animals; D001812:Blood-Brain Barrier; D002490:Central Nervous System / Q000378:metabolism*; D005732:Gangliosides / Q000378:metabolism*; D006801:Humans; D019636:Neurodegenerative Diseases / Q000378:metabolism* / Q000473:pathology*; D017510:Protein Folding
chemical_list: D005732:Gangliosides
keywords: antiganglioside antibody; gangliosides; lipid rafts; neurodegenerative diseases; treatment