Article
Pathogenic mutation of UBQLN2 impairs its interaction with UBXD8 and disrupts endoplasmic reticulum-associated protein degradation.
Journal of neurochemistry - 1 Apr 2014
Xia Yuxing, Yan Linda H, Huang Bo, Liu Mujun, Liu Xionghao, Huang Cao
Abstract excerpt
Protein aggregation is a common feature of several neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration. How protein aggregates are formed and contribute to neurodegeneration, however, is not clear. Mutation of Ubiquilin 2 (UBQLN2) has recently been linked to ALS and frontotemporal lobar degeneration. Therefore, we examined the effect of ALS-linked UBQLN2...
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