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Characterization of a PLP-overexpressing transgenic rat, a model for the connatal form of Pelizaeus–Merzbacher disease

Pelizaeus–Merzbacher disease (PMD) most frequently results from duplication of the Plp1 gene with a correlation between disease severity and increasing copy number of the gene. Animal models of PMD, in particular those overexpressing the Plp1 gene, have been sought in attempts to provide systems in...

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Principais autores: Joshua A. Mayer, Eric C. Larsen, Yoichi Kondo, Ian D. Duncan
Format: Artigo
Sprog:Inglês
Udgivet: Elsevier 2011-11-01
Serier:Neurobiology of Disease
Fag:
Pelizaeus–Merzbacher disease
Proteolipid protein
Myelination
Plp1
Apoptosis
Connatal PMD
Online adgang:http://www.sciencedirect.com/science/article/pii/S0969996111002336
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