Cargando…

Abnormal triaging of misfolded proteins by adult neuronal ceroid lipofuscinosis-associated DNAJC5/CSPα mutants causes lipofuscin accumulation

Mutations in DNAJC5/CSPα are associated with adult neuronal ceroid lipofuscinosis (ANCL), a dominant-inherited neurodegenerative disease featuring lysosome-derived autofluorescent storage materials (AFSMs) termed lipofuscin. Functionally, DNAJC5 has been implicated in chaperoning synaptic proteins a...

Descripción completa

Detalles Bibliográficos
Autores principales: Lee, Juhyung, Xu, Yue, Saidi, Layla, Xu, Miao, Zinsmaier, Konrad, Ye, Yihong
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Taylor & Francis 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9809949/
https://www.ncbi.nlm.nih.gov/pubmed/35506243
http://dx.doi.org/10.1080/15548627.2022.2065618