In vivo expression of polyglutamine-expanded huntingtin by mouse striatal astrocytes impairs glutamate transport: a correlation with Huntington's disease subjects.
Details
Serval ID
serval:BIB_60FA8F8D5FF6
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
In vivo expression of polyglutamine-expanded huntingtin by mouse striatal astrocytes impairs glutamate transport: a correlation with Huntington's disease subjects.
Journal
Human Molecular Genetics
ISSN
1460-2083 (Electronic)
ISSN-L
0964-6906
Publication state
Published
Issued date
2010
Volume
19
Number
15
Pages
3053-3067
Language
english
Notes
Publication types: Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov'tPublication Status: ppublish
Abstract
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 contribution of astrocytes in HD in vivo, we developed a novel mouse model using lentiviral vectors that results in selective expression of mHtt into striatal astrocytes. Astrocytes expressing mHtt developed a progressive phenotype of reactive astrocytes that was characterized by a marked decreased expression of both glutamate transporters, GLAST and GLT-1, and of glutamate uptake. These effects were associated with neuronal dysfunction, as observed by a reduction in DARPP-32 and NR2B expression. Parallel studies in brain samples from HD subjects revealed early glial fibrillary acidic protein expression in striatal astrocytes from Grade 0 HD cases. Astrogliosis was associated with morphological changes that increased with severity of disease, from Grades 0 through 4 and was more prominent in the putamen. Combined immunofluorescence showed co-localization of mHtt in astrocytes in all striatal HD specimens, inclusive of Grade 0 HD. Consistent with the findings from experimental mice, there was a significant grade-dependent decrease in striatal GLT-1 expression from HD subjects. These findings suggest that the presence of mHtt in astrocytes alters glial glutamate transport capacity early in the disease process and may contribute to HD pathogenesis.
Keywords
Aged, Amino Acid Transport System X-AG/metabolism, Animals, Astrocytes/metabolism, Astrocytes/pathology, Biological Transport, Dopamine and cAMP-Regulated Phosphoprotein 32/metabolism, Down-Regulation, Fluorescent Antibody Technique, Glial Fibrillary Acidic Protein/metabolism, Glutamic Acid/metabolism, Humans, Huntington Disease/metabolism, Huntington Disease/pathology, Lentivirus/genetics, Mice, Middle Aged, Mutant Proteins/metabolism, Neostriatum/metabolism, Neostriatum/pathology, 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
Pubmed
Web of science
Open Access
Yes
Create date
13/12/2011 16:13
Last modification date
20/08/2019 14:18