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Elevated TMEM106B levels exaggerate lipofuscin accumulation and lysosomal dysfunction in aged mice with progranulin deficiency

Mutations resulting in haploinsufficiency of progranulin (PGRN) cause frontotemporal lobar degeneration with TDP-43-positive inclusions (FTLD-TDP), a devastating neurodegenerative disease. Accumulating evidence suggest a crucial role of progranulin in maintaining proper lysosomal function during agi...

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Detalles Bibliográficos
Autores principales: Zhou, Xiaolai, Sun, Lirong, Brady, Owen Adam, Murphy, Kira A., Hu, Fenghua
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5270347/
https://www.ncbi.nlm.nih.gov/pubmed/28126008
http://dx.doi.org/10.1186/s40478-017-0412-1