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Loss of TMEM106B exacerbates C9ALS/FTD DPR pathology by disrupting autophagosome maturation

Disruption to protein homeostasis caused by lysosomal dysfunction and associated impairment of autophagy is a prominent pathology in amyotrophic lateral sclerosis and frontotemporal dementia (ALS/FTD). The most common genetic cause of ALS/FTD is a G4C2 hexanucleotide repeat expansion in C9orf72 (C9A...

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Detalles Bibliográficos
Autores principales: Bauer, Claudia S., Webster, Christopher P., Shaw, Allan C., Kok, Jannigje R., Castelli, Lydia M., Lin, Ya-Hui, Smith, Emma F., Illanes-Álvarez, Francisco, Higginbottom, Adrian, Shaw, Pamela J., Azzouz, Mimoun, Ferraiuolo, Laura, Hautbergue, Guillaume M., Grierson, Andrew J., De Vos, Kurt J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9812496/
https://www.ncbi.nlm.nih.gov/pubmed/36619668
http://dx.doi.org/10.3389/fncel.2022.1061559