Peroxisome-proliferator-activated receptor gamma coactivator 1 α contributes to dysmyelination in experimental models of Huntington's disease

• Published in: The Journal of neuroscience Vol. 31; no. 26; pp. 9544 - 9553
• Main Authors: Xiang, Zhongmin, Valenza, Marta, Cui, Libin, Leoni, Valerio, Jeong, Hyun-Kyung, Brilli, Elisa, Zhang, Jiangyang, Peng, Qi, Duan, Wenzhen, Reeves, Steven A, Cattaneo, Elena, Krainc, Dimitri
• Format: Journal Article
• Language: English
• Published: United States Society for Neuroscience 29.06.2011
• Subjects: Analysis of Variance; Animals; Blotting, Western; Brain - metabolism; Brain - pathology; Chromatin Immunoprecipitation; Demyelinating Diseases - genetics; Demyelinating Diseases - metabolism; Demyelinating Diseases - pathology; Diffusion Tensor Imaging; Disease Models, Animal; Huntington Disease - genetics; Huntington Disease - metabolism; Huntington Disease - pathology; Hydroxymethylglutaryl CoA Reductases - genetics; Hydroxymethylglutaryl CoA Reductases - metabolism; Hydroxymethylglutaryl-CoA Synthase - genetics; Hydroxymethylglutaryl-CoA Synthase - metabolism; Immunohistochemistry; Mice; Mice, Knockout; Myelin Basic Protein - genetics; Myelin Basic Protein - metabolism; Myelin Sheath - metabolism; Myelin Sheath - pathology; Oligodendroglia - metabolism; Oligodendroglia - pathology; Reverse Transcriptase Polymerase Chain Reaction; Transcription Factors - genetics; Transcription Factors - metabolism

The peroxisome-proliferator-activated receptor gamma coactivator 1 α (PGC1α) has been implicated in the pathogenesis of several neurodegenerative disorders, including Huntington's disease (HD). Recent data demonstrating white matter abnormalities in PGC1α knock-out (KO) mice prompted us to exam...