Increased translation as a novel pathogenic mechanism in Huntington’s disease

Author:

Creus-Muncunill Jordi123,Badillos-Rodríguez Raquel123,Garcia-Forn Marta123,Masana Mercè123,Garcia-Díaz Barriga Gerardo123,Guisado-Corcoll Anna123,Alberch Jordi123,Malagelada Cristina1,Delgado-García José M4,Gruart Agnès4,Pérez-Navarro Esther123

Affiliation:

1. Department of Biomedical Sciences, Faculty of Medicine and Health Sciences, Institute of Neurosciences, University of Barcelona, Barcelona, Catalonia

2. Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Catalonia

3. Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Spain

4. Division of Neurosciences, Pablo de Olavide University, Seville, Spain

Abstract

See Brouillet and Merienne (doi:10.1093/brain/awz274) for a scientific commentary on this article.Creus-Muncunill et al. report that protein translation is altered in the striatum of Huntington’s disease mouse models, with upregulated expression of proteins from ribosomal and oxidative phosphorylation pathways. Pharmacological normalization of protein translation in R6/1 mice ameliorates motor disturbances and normalizes ribosomal content in the striatum.

Funder

Ministerio de Economia y Competitividad, Spain

Fundación Tatiana Pérez de Guzmán el Bueno

Publisher

Oxford University Press (OUP)

Subject

Clinical Neurology

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