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Comment
. 2016 Apr 15;35(8):798-800.
doi: 10.15252/embj.201694117. Epub 2016 Mar 11.

ER strikes again: Proteostasis Dysfunction In ALS

Niran Maharjan  1 , Smita Saxena  1
Affiliations
Comment

ER strikes again: Proteostasis Dysfunction In ALS

Niran Maharjan et al. EMBO J. .

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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Figures

Figure 1
Figure 1. Proposed mechanism by which the suboptimal functioning of ERp57 impairs motor neuron proteostasis and causes ALS‐like symptoms
See this image and copyright information in PMC

Comment on

  • ALS-linked protein disulfide isomerase variants cause motor dysfunction.
    Woehlbier U, Colombo A, Saaranen MJ, Pérez V, Ojeda J, Bustos FJ, Andreu CI, Torres M, Valenzuela V, Medinas DB, Rozas P, Vidal RL, Lopez-Gonzalez R, Salameh J, Fernandez-Collemann S, Muñoz N, Matus S, Armisen R, Sagredo A, Palma K, Irrazabal T, Almeida S, Gonzalez-Perez P, Campero M, Gao FB, Henny P, van Zundert B, Ruddock LW, Concha ML, Henriquez JP, Brown RH, Hetz C. Woehlbier U, et al. EMBO J. 2016 Apr 15;35(8):845-65. doi: 10.15252/embj.201592224. Epub 2016 Feb 11. EMBO J. 2016. PMID: 26869642 Free PMC article.

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