ER stress and the unfolded protein response in neurodegeneration

C Hetz, S Saxena - Nature Reviews Neurology, 2017 - nature.com
The clinical manifestation of neurodegenerative diseases is initiated by the selective
alteration in the functionality of distinct neuronal populations. The pathology of many …

Folding and misfolding of human membrane proteins in health and disease: from single molecules to cellular proteostasis

JT Marinko, H Huang, WD Penn, JA Capra… - Chemical …, 2019 - ACS Publications
Advances over the past 25 years have revealed much about how the structural properties of
membranes and associated proteins are linked to the thermodynamics and kinetics of …

A selective ER‐phagy exerts procollagen quality control via a Calnexin‐FAM 134B complex

A Forrester, C De Leonibus, P Grumati, E Fasana… - The EMBO …, 2019 - embopress.org
Autophagy is a cytosolic quality control process that recognizes substrates through receptor‐
mediated mechanisms. Procollagens, the most abundant gene products in Metazoa, are …

Calnexin, more than just a molecular chaperone

T Paskevicius, RA Farraj, M Michalak, LB Agellon - Cells, 2023 - mdpi.com
Calnexin is a type I integral endoplasmic reticulum (ER) membrane protein with an N-
terminal domain that resides in the lumen of the ER and a C-terminal domain that extends …

Adapting the proteostasis capacity to sustain brain healthspan

C Hetz - Cell, 2021 - cell.com
Sustaining neuronal proteostasis during the course of our life is a central aspect required for
brain function. The dynamic nature of synaptic composition and abundance is a requisite to …

[HTML][HTML] A sweet code for glycoprotein folding

JJ Caramelo, AJ Parodi - FEBS letters, 2015 - Elsevier
Glycoprotein synthesis is initiated in the endoplasmic reticulum (ER) lumen upon transfer of
a glycan (Glc 3 Man 9 GlcNAc 2) from a lipid derivative to Asn residues (N-glycosylation). N …

ER proteostasis control of neuronal physiology and synaptic function

G Martínez, S Khatiwada, M Costa-Mattioli… - Trends in neurosciences, 2018 - cell.com
Neuronal proteostasis is maintained by the dynamic integration of different processes that
regulate the synthesis, folding, quality control, and localization of proteins. The endoplasmic …

Palmitoylation is the switch that assigns calnexin to quality control or ER Ca2+ signaling

EM Lynes, A Raturi, M Shenkman… - Journal of cell …, 2013 - journals.biologists.com
The palmitoylation of calnexin serves to enrich calnexin on the mitochondria-associated
membrane (MAM). Given a lack of information on the significance of this finding, we have …

[HTML][HTML] The cell stress machinery and retinal degeneration

D Athanasiou, M Aguilà, D Bevilacqua, SS Novoselov… - FEBS letters, 2013 - Elsevier
Retinal degenerations are a group of clinically and genetically heterogeneous disorders
characterised by progressive loss of vision due to neurodegeneration. The retina is a highly …

ALS‐linked protein disulfide isomerase variants cause motor dysfunction

U Woehlbier, A Colombo, MJ Saaranen, V Pérez… - The EMBO …, 2016 - embopress.org
Disturbance of endoplasmic reticulum (ER) proteostasis is a common feature of amyotrophic
lateral sclerosis (ALS). Protein disulfide isomerases (PDI s) are ER foldases identified as …