The mouse C9ORF72 ortholog is enriched in neurons known to degenerate in ALS and FTD

Using transgenic animals harboring a targeted LacZ insertion, we studied the expression pattern of the C9ORF72 mouse ortholog. Unlike most genes mutated in ALS, which are ubiquitously expressed, the C9ORF72-ortholog was most highly transcribed in the neuronal populations sensitive to degeneration in...

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Detalles Bibliográficos
Autores principales: Suzuki, Naoki, Maroof, Asif, Merkle, Florian T, Koszka, Kathryn, Intoh, Atsushi, Armstrong, Ian, Moccia, Rob, Davis-Dusenbery, Brandi N, Eggan, Kevin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: 2013
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4397902/
https://www.ncbi.nlm.nih.gov/pubmed/24185425
http://dx.doi.org/10.1038/nn.3566
Descripción
Sumario:Using transgenic animals harboring a targeted LacZ insertion, we studied the expression pattern of the C9ORF72 mouse ortholog. Unlike most genes mutated in ALS, which are ubiquitously expressed, the C9ORF72-ortholog was most highly transcribed in the neuronal populations sensitive to degeneration in ALS and FTD. Thus, our study provides a potential explanation for the cell type specificity of neuronal degeneration caused by C9ORF72 mutations.

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