ER strikes again: Proteostasis Dysfunction In ALS

作者:Maharjan Niran; Saxena Smita
来源:The EMBO Journal, 2016, 35(8): 798-800.
DOI:10.15252/embj.201694117

摘要

The precise contribution of endoplasmic reticulum (ER) chaperone protein disulfide isomerase (PDI) variants in human amyotrophic lateral sclerosis (ALS) patients to the pathogenesis of ALS remained unclear. In the present study, Woehlbier etal (2016) demonstrated that these PDI variants are capable of altering motor neuron morphology, impairing the expression of synaptic proteins, and compromising neuromuscular junction (NMJ) integrity.

  • 出版日期2016-4-15