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Ganglioside Synthase Knockout Reduces Prion Disease Incubation Time in Mouse Models

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Title: Ganglioside Synthase Knockout Reduces Prion Disease Incubation Time in Mouse Models
Authors: Kobayashi, Atsushi Browse this author
Qi, Zechen Browse this author
Shimazaki, Taishi Browse this author
Munesue, Yoshiko Browse this author
Miyamoto, Tomomi Browse this author
Isoda, Norikazu Browse this author
Sawa, Hirofumi Browse this author
Aoshima, Keisuke Browse this author
Kimura, Takashi Browse this author
Mohri, Shirou Browse this author
Kitamoto, Tetsuyuki Browse this author
Yamashita, Tadashi Browse this author
Miyoshi, Ichiro Browse this author
Issue Date: Mar-2019
Publisher: Elsevier
Journal Title: The American Journal of Pathology
Volume: 189
Issue: 3
Start Page: 677
End Page: 686
Publisher DOI: 10.1016/j.ajpath.2018.11.009
Abstract: Localization of the abnormal and normal isoforms of prion proteins to detergent-resistant membrane microdomains, lipid rafts, is important for the conformational conversion. Lipid rafts are enriched in sialic acid-containing glycosphingolipids (namely, gangliosides). Alteration in the ganglioside composition of lipid rafts can affect the localization of lipid raft-associated proteins. To investigate the role of gangliosides in the pathogenesis of prion diseases, we performed intracerebral transmission study of a scrapie prion strain Chandler and a Gerstmann-Straussler-Scheinker syndrome prion strain Fukuoka-1 using various knockout mouse strains ablated with ganglioside synthase gene (ie, GD2/GM2 synthase, GD3 synthase, or GM3 synthase). After challenge with the Chandler strain, GD2/GM2 synthase knockout mice showed 20% reduction of incubation time, reduced prion protein deposition in the brain with attenuated glial reactions, and reduced localization of prion proteins to lipid rafts. These results raise the possibility that the gangliosides may have an important role in prion disease pathogenesis by affecting the localization of prion proteins to lipid rafts.
Rights: ©2019, Elsevier. Licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International
Type: article (author version)
Appears in Collections:獣医学院・獣医学研究院 (Graduate School of Veterinary Medicine / Faculty of Veterinary Medicine) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)

Submitter: 小林 篤史

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