Restoring brain cholesterol turnover improves autophagy and has therapeutic potential in mouse models of spinocerebellar ataxia

• Published in: Acta neuropathologica Vol. 138; no. 5; pp. 837 - 858
• Main Authors: Nóbrega, Clévio, Mendonça, Liliana, Marcelo, Adriana, Lamazière, Antonin, Tomé, Sandra, Despres, Gaetan, Matos, Carlos A., Mechmet, Fatich, Langui, Dominique, den Dunnen, Wilfred, de Almeida, Luis Pereira, Cartier, Nathalie, Alves, Sandro
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.11.2019; Springer; Springer Nature B.V
• Subjects: Analysis; Animal models; Ataxia; Ataxin; Atrophy; Autophagy; Cell number; Cerebellum; Cholesterol; Cytochrome P-450; Genetic engineering; Genotype & phenotype; Health aspects; Hydroxylase; Lipid metabolism; Medical research; Medicine; Medicine & Public Health; Medicine, Experimental; Medicine, Preventive; Metabolism; Nervous system diseases; Neurodegeneration; Neurodegenerative diseases; Neurons; Neurophysiology; Neuroprotection; Neurosciences; Original Paper; Pathology; Phagocytosis; Phenotypes; Physiological aspects; Preventive health services; Protein interaction; Rodents; Spinocerebellar ataxia; Therapeutic applications; Ataxia; SCA animal models; Cholesterol metabolism; 24-Cholesterol hydroxylase; Autophagy; SCA patients

Spinocerebellar ataxias (SCAs) are devastating neurodegenerative disorders for which no curative or preventive therapies are available. Deregulation of brain cholesterol metabolism and impaired brain cholesterol turnover have been associated with several neurodegenerative diseases. SCA3 or Machado–J...