Atxn2-CAG100-KnockIn mouse spinal cord shows progressive TDP43 pathology associated with cholesterol biosynthesis suppression

Atxn2-CAG100-KnockIn mouse spinal cord shows progressive TDP43 pathology associated with cholesterol biosynthesis suppression

Large polyglutamine expansions in Ataxin-2 (ATXN2) cause multi-system nervous atrophy in Spinocerebellar Ataxia type 2 (SCA2). Intermediate size expansions carry a risk for selective motor neuron degeneration, known as Amyotrophic Lateral Sclerosis (ALS). Conversely, the depletion of ATXN2 prevents...

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Bibliographic Details
Main Authors: Júlia Canet-Pons, Nesli-Ece Sen, Aleksandar Arsović, Luis-Enrique Almaguer-Mederos, Melanie V. Halbach, Jana Key, Claudia Döring, Anja Kerksiek, Gina Picchiarelli, Raphaelle Cassel, Frédérique René, Stéphane Dieterlé, Nina V. Fuchs, Renate König, Luc Dupuis, Dieter Lütjohann, Suzana Gispert, Georg Auburger
Format: Article
Language:English
Published: Elsevier 2021-05-01
Series:Neurobiology of Disease
Subjects:
Olivo-ponto-cerebellar atrophy
Tauopathy
Neuroinflammation
Demyelination
Leukoencephalopathy
Steroidogenesis
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996121000383

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http://www.sciencedirect.com/science/article/pii/S0969996121000383

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