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Misalignment of PLP/DM20 Transmembrane Domains Determines Protein Misfolding in Pelizaeus–Merzbacher Disease

A large number of genetic diseases have been associated with truncated or misfolded membrane proteins trapped in the endoplasmic reticulum (ER). In the ER, they activate the unfolded protein response, which can trigger cell death. Hence, a better understanding of protein misfolding features might he...

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Publicat a:J Neurosci
Autors principals: Dhaunchak, Ajit Singh, Colman, David R., Nave, Klaus-Armin
Format: Artigo
Idioma:Inglês
Publicat: Society for Neuroscience 2011
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Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC6623585/
https://ncbi.nlm.nih.gov/pubmed/22016529
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1523/JNEUROSCI.2097-11.2011
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