ER strikes again: Proteostasis Dysfunction In ALS |
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| Authors: | Niran Maharjan Smita Saxena |
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| Affiliation: | Institute of Cell Biology, University of Bern, Bern, Switzerland |
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| Abstract: | 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 et al ( 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. |
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