ALS ‐linked protein disulfide isomerase variants cause motor dysfunction
نویسندگان
چکیده
منابع مشابه
ALS-linked protein disulfide isomerase variants cause motor dysfunction.
Disturbance of endoplasmic reticulum (ER) proteostasis is a common feature of amyotrophic lateral sclerosis (ALS). Protein disulfide isomerases (PDIs) areERfoldases identified as possibleALSbiomarkers, as well as neuroprotective factors. However, no functional studies have addressed their impact on the disease process. Here, we functionally characterized fourALS-linked mutations recently identi...
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Protein disulfide isomerase (PDI) is an oxidoreductase assisting oxidative protein folding in the endoplasmic reticulum of all types of cells, including neurons and glia. In neurodegenerative disorders, such as amyotrophic lateral sclerosis (ALS), up-regulation of PDI is an important part of unfolded protein response (UPR) that is thought to represent an adaption reaction and thereby protect th...
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ژورنال
عنوان ژورنال: The EMBO Journal
سال: 2016
ISSN: 0261-4189,1460-2075
DOI: 10.15252/embj.201592224