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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...
Autores principales: | Lee, Juhyung, Xu, Yue, Saidi, Layla, Xu, Miao, Zinsmaier, Konrad, Ye, Yihong |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Taylor & Francis
2022
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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 |
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