Polymerogenic neuroserpin causes mitochondrial alterations and activates NFκB but not the UPR in a neuronal model of neurodegeneration FENIB

The neurodegenerative condition FENIB (familiar encephalopathy with neuroserpin inclusion bodies) is caused by heterozygous expression of polymerogenic mutant neuroserpin (NS), with polymer deposition within the endoplasmic reticulum (ER) of neurons. We generated transgenic neural progenitor cells (...

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Detalles Bibliográficos
Autores principales: D’Acunto, E., Gianfrancesco, L., Serangeli, I., D’Orsi, M., Sabato, V., Guadagno, N. A., Bhosale, G., Caristi, S., Failla, A. V., De Jaco, A., Cacci, E., Duchen, M. R., Lupo, G., Galliciotti, G., Miranda, E.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer International Publishing 2022
Materias:
Original Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9304071/
https://www.ncbi.nlm.nih.gov/pubmed/35864382
http://dx.doi.org/10.1007/s00018-022-04463-3

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