Mouse model of multiple system atrophy alpha-synuclein expression in oligodendrocytes causes glial and neuronal degeneration.
Neuron
; 45(6): 847-59, 2005 Mar 24.
Article
em En
| MEDLINE
| ID: mdl-15797547
ABSTRACT
Transgenic (Tg) mice overexpressing human wild-type alpha-synuclein in oligodendrocytes under the control of the 2,' 3'-cyclic nucleotide 3'-phosphodiesterase (CNP) promoter are shown here to recapitulate features of multiple system atrophy (MSA), including the accumulation of filamentous human alpha-synuclein aggregates in oligodendrocytes linked to their degeneration and autophagocytosis of myelin. Significantly, endogenous mouse alpha-synuclein also accumulated in normal and degenerating axons and axon terminals in association with oligodendroglia and neuron loss and slowly progressive motor impairments. Our studies demonstrate that overexpression of alpha-synuclein in oligodendrocytes of mice results in MSA-like degeneration in the CNS and that alpha-synuclein inclusions in oligodendrocytes participate in the degeneration of neurons in MSA.
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Base de dados:
MEDLINE
Assunto principal:
Degeneração Walleriana
/
Oligodendroglia
/
Neuroglia
/
Atrofia de Múltiplos Sistemas
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Proteínas do Tecido Nervoso
/
Neurônios
Tipo de estudo:
Etiology_studies
Idioma:
En
Ano de publicação:
2005
Tipo de documento:
Article