Familial CJD associated PrP mutants within transmembrane region induced Ctm-PrP retention in ER and triggered apoptosis by ER stress in SH-SY5Y cells.
<h4>Background</h4>Genetic prion diseases are linked to point and inserted mutations in the prion protein (PrP) gene that are presumed to favor conversion of the cellular isoform of PrP (PrP(C)) to the pathogenic one (PrP(Sc)). The pathogenic mechanisms and the subcellular sites of the c...
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Autores principales: | Xin Wang, Qi Shi, Kun Xu, Chen Gao, Cao Chen, Xiao-Li Li, Gui-Rong Wang, Chan Tian, Jun Han, Xiao-Ping Dong |
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Formato: | article |
Lenguaje: | EN |
Publicado: |
Public Library of Science (PLoS)
2011
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Materias: | |
Acceso en línea: | https://doaj.org/article/13ac0b150d884dd094fbd9aa6e9953a6 |
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