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Soluble and insoluble dipeptide repeat protein measurements in C9orf72-frontotemporal dementia brains show regional differential solubility and correlation of poly-GR with clinical severity
A C9orf72 repeat expansion is the most common genetic cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis. One of the suggested pathomechanisms is toxicity from dipeptide repeat proteins (DPRs), which are generated via unconventional translation of sense and antisense repeat tra...
Autores principales: | Quaegebeur, Annelies, Glaria, Idoia, Lashley, Tammaryn, Isaacs, Adrian M. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7650212/ https://www.ncbi.nlm.nih.gov/pubmed/33168090 http://dx.doi.org/10.1186/s40478-020-01036-y |
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