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 …

The biology of proteostasis in aging and disease

J Labbadia, RI Morimoto - Annual review of biochemistry, 2015 - annualreviews.org
Loss of protein homeostasis (proteostasis) is a common feature of aging and disease that is
characterized by the appearance of nonnative protein aggregates in various tissues. Protein …

Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis

SC Ling, M Polymenidou, DW Cleveland - Neuron, 2013 - cell.com
Breakthrough discoveries identifying common genetic causes for amyotrophic lateral
sclerosis (ALS) and frontotemporal dementia (FTD) have transformed our view of these …

There's something wrong with my MAM; the ER–mitochondria axis and neurodegenerative diseases

S Paillusson, R Stoica, P Gomez-Suaga… - Trends in …, 2016 - cell.com
Alzheimer's disease (AD), Parkinson's disease (PD), and amyotrophic lateral sclerosis with
associated frontotemporal dementia (ALS/FTD) are major neurodegenerative diseases for …

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 …

VAP, a versatile access point for the endoplasmic reticulum: review and analysis of FFAT-like motifs in the VAPome

SE Murphy, TP Levine - Biochimica et Biophysica Acta (BBA)-Molecular and …, 2016 - Elsevier
Dysfunction of VAMP-associated protein (VAP) is associated with neurodegeneration, both
Amyotrophic Lateral Sclerosis and Parkinson's disease. Here we summarize what is known …

VAPB interacts with the mitochondrial protein PTPIP51 to regulate calcium homeostasis

KJ De Vos, GM Morotz, R Stoica… - Human molecular …, 2012 - academic.oup.com
A proline to serine substitution at position 56 in the gene encoding vesicle-associated
membrane protein-associated protein B (VAPB) causes some dominantly inherited familial …

Metabolic implications of organelle–mitochondria communication

I Gordaliza‐Alaguero, C Cantó, A Zorzano - EMBO reports, 2019 - embopress.org
Cellular organelles are not static but show dynamism—a property that is likely relevant for
their function. In addition, they interact with other organelles in a highly dynamic manner. In …

XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy

C Hetz, P Thielen, S Matus, M Nassif… - Genes & …, 2009 - genesdev.cshlp.org
Mutations in superoxide dismutase-1 (SOD1) cause familial amyotrophic lateral sclerosis
(fALS). Recent evidence implicates adaptive responses to endoplasmic reticulum (ER) …