Endoplasmic reticulum stress and unfolded protein response in neurodegenerative diseases

R Ghemrawi, M Khair - International journal of molecular sciences, 2020 - mdpi.com
The endoplasmic reticulum (ER) is an important organelle involved in protein quality control
and cellular homeostasis. The accumulation of unfolded proteins leads to an ER stress …

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 …

Motor neuron susceptibility in ALS/FTD

AMG Ragagnin, S Shadfar, M Vidal… - Frontiers in …, 2019 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the
death of both upper and lower motor neurons (MNs) in the brain, brainstem and spinal cord …

[HTML][HTML] Axonal transport defects and neurodegeneration: molecular mechanisms and therapeutic implications

W Guo, KS Dittlau, L Van Den Bosch - Seminars in cell & developmental …, 2020 - Elsevier
Because of the extremely polarized morphology, the proper functioning of neurons largely
relies on the efficient cargo transport along the axon. Axonal transport defects have been …

Endoplasmic reticulum proteostasis impairment in aging

G Martínez, C Duran‐Aniotz, F Cabral‐Miranda… - Aging cell, 2017 - Wiley Online Library
Perturbed neuronal proteostasis is a salient feature shared by both aging and protein
misfolding disorders. The proteostasis network controls the health of the proteome by …

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 …

CNS-derived extracellular vesicles from superoxide dismutase 1 (SOD1) G93A ALS mice originate from astrocytes and neurons and carry misfolded SOD1

JM Silverman, D Christy, CC Shyu, KM Moon… - Journal of Biological …, 2019 - ASBMB
Extracellular vesicles (EVs) are secreted by myriad cells in culture and also by unicellular
organisms, and their identification in mammalian fluids suggests that EV release also occurs …

Targeting PERK signaling with the small molecule GSK2606414 prevents neurodegeneration in a model of Parkinson's disease

G Mercado, V Castillo, P Soto, N López, JM Axten… - Neurobiology of …, 2018 - Elsevier
Parkinson's disease (PD) is the second most common neurodegenerative disorder, leading
to the progressive decline of motor control due to the loss of dopaminergic neurons in the …

Protein disulfide isomerases: Redox connections in and out of the endoplasmic reticulum

AIS Moretti, FRM Laurindo - Archives of biochemistry and biophysics, 2017 - Elsevier
Protein disulfide isomerases are thiol oxidoreductase chaperones from thioredoxin
superfamily. As redox folding catalysts from the endoplasmic reticulum (ER), their roles in …

CRISPR/Cas9-mediated targeted gene correction in amyotrophic lateral sclerosis patient iPSCs

L Wang, F Yi, L Fu, J Yang, S Wang, Z Wang… - Protein & …, 2017 - academic.oup.com
Amyotrophic lateral sclerosis (ALS) is a complex neurodegenerative disease with cellular
and molecular mechanisms yet to be fully described. Mutations in a number of genes …