Differences in endoplasmic-reticulum quality control determine the cellular response to disease-associated mutants of proteolipid protein

Missense mutations in human PLP1, the gene encoding myelin proteolipid protein (PLP), cause dysmyelinating Pelizaeus-Merzbacher disease of varying severity. Although disease pathology has been linked to retention of misfolded PLP in the endoplasmic reticulum (ER) and induction of the unfolded protei...

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Bibliographic Details
Main Authors: Roboti, Peristera, Swanton, Eileithyia, High, Stephen
Format: Artigo
Language:Inglês
Published: Company of Biologists 2009
Subjects:
Research Article
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC2773193/
https://ncbi.nlm.nih.gov/pubmed/19825935
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1242/jcs.055160
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