Frontotemporal dementia caused by CHMP2B mutation is characterised by neuronal lysosomal storage pathology

Mutations in the charged multivesicular body protein 2B (CHMP2B) cause frontotemporal dementia (FTD). We report that mice which express FTD-causative mutant CHMP2B at physiological levels develop a novel lysosomal storage pathology characterised by large neuronal autofluorescent aggregates. The aggr...

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Detalles Bibliográficos
Autores principales: Clayton, Emma L., Mizielinska, Sarah, Edgar, James R., Nielsen, Troels Tolstrup, Marshall, Sarah, Norona, Frances E., Robbins, Miranda, Damirji, Hana, Holm, Ida E., Johannsen, Peter, Nielsen, Jørgen E., Asante, Emmanuel A., Collinge, John, Isaacs, Adrian M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Berlin Heidelberg 2015
Materias:
Original Paper
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4575387/
https://www.ncbi.nlm.nih.gov/pubmed/26358247
http://dx.doi.org/10.1007/s00401-015-1475-3

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