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ER stress and the unfolded protein response in neurodegeneration
The clinical manifestation of neurodegenerative diseases is initiated by the selective
alteration in the functionality of distinct neuronal populations. The pathology of many …
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
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 …
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 …
mediated mechanisms. Procollagens, the most abundant gene products in Metazoa, are …
Calnexin, more than just a molecular chaperone
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 …
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 …
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 …
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 …
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 …
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 …
characterised by progressive loss of vision due to neurodegeneration. The retina is a highly …
ALS‐linked protein disulfide isomerase variants cause motor dysfunction
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 …
lateral sclerosis (ALS). Protein disulfide isomerases (PDI s) are ER foldases identified as …