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Loss of CLN7 results in depletion of soluble lysosomal proteins and impaired mTOR reactivation

Defects in the MFSD8 gene encoding the lysosomal membrane protein CLN7 lead to CLN7 disease, a neurodegenerative lysosomal storage disorder belonging to the group of neuronal ceroid lipofuscinoses. Here, we have performed a SILAC-based quantitative analysis of the lysosomal proteome using Cln7-defic...

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Detalles Bibliográficos
Autores principales: Danyukova, Tatyana, Ariunbat, Khandsuren, Thelen, Melanie, Brocke-Ahmadinejad, Nahal, Mole, Sara E, Storch, Stephan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5932567/
https://www.ncbi.nlm.nih.gov/pubmed/29514215
http://dx.doi.org/10.1093/hmg/ddy076