Improving clinical trial outcomes in amyotrophic lateral sclerosis

MC Kiernan, S Vucic, K Talbot, CJ McDermott… - Nature Reviews …, 2021 - nature.com
Individuals who are diagnosed with amyotrophic lateral sclerosis (ALS) today face the same
historically intransigent problem that has existed since the initial description of the disease in …

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 …

Decoding ALS: from genes to mechanism

JP Taylor, RH Brown Jr, DW Cleveland - Nature, 2016 - nature.com
Amyotrophic lateral sclerosis (ALS) is a progressive and uniformly fatal neurodegenerative
disease. A plethora of genetic factors have been identified that drive the degeneration of …

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 …

Targeting autophagy, oxidative stress, and ER stress for neurodegenerative disease treatment

Y Esmaeili, Z Yarjanli, F Pakniya, E Bidram… - Journal of Controlled …, 2022 - Elsevier
Protein homeostasis is a vital process for cell function and, therefore, disruption of the
molecular mechanisms involved in this process, such as autophagy, may contribute to …

Proteotoxic stress and the ubiquitin proteasome system

R Kandel, J Jung, S Neal - Seminars in cell & developmental biology, 2024 - Elsevier
The ubiquitin proteasome system maintains protein homeostasis by regulating the
breakdown of misfolded proteins, thereby preventing misfolded protein aggregates. The …

The role of endoplasmic reticulum stress in human pathology

SA Oakes, FR Papa - Annual Review of Pathology: Mechanisms …, 2015 - annualreviews.org
Numerous genetic and environmental insults impede the ability of cells to properly fold and
posttranslationally modify secretory and transmembrane proteins in the endoplasmic …

The unfolded protein response: controlling cell fate decisions under ER stress and beyond

C Hetz - Nature reviews Molecular cell biology, 2012 - nature.com
Protein-folding stress at the endoplasmic reticulum (ER) is a salient feature of specialized
secretory cells and is also involved in the pathogenesis of many human diseases. ER stress …

Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases

C Hetz, B Mollereau - Nature Reviews Neuroscience, 2014 - nature.com
The unfolded protein response (UPR) is a homeostatic mechanism by which cells regulate
levels of misfolded proteins in the endoplasmic reticulum (ER). Although it is well …

[HTML][HTML] Pathological roles of MAPK signaling pathways in human diseases

EK Kim, EJ Choi - Biochimica et Biophysica Acta (BBA)-Molecular Basis …, 2010 - Elsevier
The mammalian family of mitogen-activated protein kinases (MAPKs) includes extracellular
signal-regulated kinase (ERK), p38, and c-Jun NH2-terminal kinase (JNK), with each MAPK …