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Progranulin reduces insoluble TDP-43 levels, slows down axonal degeneration and prolongs survival in mutant TDP-43 mice
BACKGROUND: TAR DNA binding protein 43 (TDP-43) is the main disease protein in most patients with amyotrophic lateral sclerosis (ALS) and about 50% of patients with frontotemporal dementia (FTD). TDP-43 pathology is not restricted to patients with missense mutations in TARDBP, the gene encoding TDP-...
Autores principales: | Beel, Sander, Herdewyn, Sarah, Fazal, Raheem, De Decker, Mathias, Moisse, Matthieu, Robberecht, Wim, Van Den Bosch, Ludo, Van Damme, Philip |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6192075/ https://www.ncbi.nlm.nih.gov/pubmed/30326935 http://dx.doi.org/10.1186/s13024-018-0288-y |
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