In vivo expression of polyglutamine-expanded huntingtin by mouse striatal astrocytes impairs glutamate transport: a correlation with Huntington's disease subjects

• Published in: Human molecular genetics Vol. 19; no. 15; pp. 3053 - 3067
• Main Authors: Faideau, Mathilde, Kim, Jinho, Cormier, Kerry, Gilmore, Richard, Welch, Mackenzie, Auregan, Gwennaelle, Dufour, Noelle, Guillermier, Martine, Brouillet, Emmanuel, Hantraye, Philippe, Déglon, Nicole, Ferrante, Robert J., Bonvento, Gilles
• Format: Journal Article
• Language: English
• Published: Oxford Oxford University Press 01.08.2010
• Subjects: Aged; Amino Acid Transport System X-AG - metabolism; Animals; Astrocytes - metabolism; Astrocytes - pathology; Biological and medical sciences; Biological Transport; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Dopamine and cAMP-Regulated Phosphoprotein 32 - metabolism; Down-Regulation; Fluorescent Antibody Technique; Fundamental and applied biological sciences. Psychology; Genetics of eukaryotes. Biological and molecular evolution; Glial Fibrillary Acidic Protein - metabolism; Glutamic Acid - metabolism; Humans; Huntington Disease - metabolism; Huntington Disease - pathology; Lentivirus - genetics; Medical sciences; Mice; Middle Aged; Molecular and cellular biology; Mutant Proteins - metabolism; Neostriatum - metabolism; Neostriatum - pathology; Neurology; Neurons - metabolism; Neurons - pathology; Peptides - metabolism; Phenotype; Receptors, N-Methyl-D-Aspartate - metabolism; Serotonin Plasma Membrane Transport Proteins - metabolism; Time Factors; Trinucleotide Repeat Expansion - genetics; Nervous system diseases; Correlation; Huntingtin; Neuroglia; Rodentia; Huntington disease; Central nervous system; Astrocyte; Glutamate; Genetic disease; Cerebral disorder; In vivo; Vertebrata; Mammalia; Mouse; Central nervous system disease; Excitatory aminoacid; Neurotransmitter; Genetics; Degenerative disease; Extrapyramidal syndrome

Huntington's disease (HD) is a neurodegenerative disorder previously thought to be of primary neuronal origin, despite ubiquitous expression of mutant huntingtin (mHtt). We tested the hypothesis that mHtt expressed in astrocytes may contribute to the pathogenesis of HD. To better understand the...