[HTML][HTML] The role of mitochondria in amyotrophic lateral sclerosis
Mitochondria are unique organelles that are essential for a variety of cellular processes
including energy metabolism, calcium homeostasis, lipid biosynthesis, and apoptosis …
including energy metabolism, calcium homeostasis, lipid biosynthesis, and apoptosis …
Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis
Breakthrough discoveries identifying common genetic causes for amyotrophic lateral
sclerosis (ALS) and frontotemporal dementia (FTD) have transformed our view of these …
sclerosis (ALS) and frontotemporal dementia (FTD) have transformed our view of these …
Protein aggregation in amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the
aggregation of ubiquitinated proteins in affected motor neurons. Recent studies have …
aggregation of ubiquitinated proteins in affected motor neurons. Recent studies have …
[HTML][HTML] TDP-43 proteinopathy and ALS: insights into disease mechanisms and therapeutic targets
Therapeutic options for patients with amyotrophic lateral sclerosis (ALS) are currently
limited. However, recent studies show that almost all cases of ALS, as well as tau-negative …
limited. However, recent studies show that almost all cases of ALS, as well as tau-negative …
[HTML][HTML] Neurobiology of axonal transport defects in motor neuron diseases: Opportunities for translational research?
KJ De Vos, M Hafezparast - Neurobiology of disease, 2017 - Elsevier
Intracellular trafficking of cargoes is an essential process to maintain the structure and
function of all mammalian cell types, but especially of neurons because of their extreme …
function of all mammalian cell types, but especially of neurons because of their extreme …
[HTML][HTML] Stress granule mediated protein aggregation and underlying gene defects in the FTD-ALS spectrum
Y Baradaran-Heravi, C Van Broeckhoven… - Neurobiology of …, 2020 - Elsevier
Stress granules (SGs) are dynamic membraneless compartments composed out of RNA-
binding proteins (RBPs) and RNA molecules that assemble temporarily to allow the cell to …
binding proteins (RBPs) and RNA molecules that assemble temporarily to allow the cell to …
The pathobiology of TDP-43 C-terminal fragments in ALS and FTLD
BA Berning, AK Walker - Frontiers in neuroscience, 2019 - frontiersin.org
During neurodegenerative disease, the multifunctional RNA-binding protein TDP-43
undergoes a vast array of post-translational modifications, including phosphorylation …
undergoes a vast array of post-translational modifications, including phosphorylation …
Clinical perspective on oxidative stress in sporadic amyotrophic lateral sclerosis
E D'Amico, P Factor-Litvak, RM Santella… - Free radical biology and …, 2013 - Elsevier
Sporadic amyotrophic lateral sclerosis (ALS) is one of the most devastating neurological
diseases; most patients die within 3 to 4 years after symptom onset. Oxidative stress is a …
diseases; most patients die within 3 to 4 years after symptom onset. Oxidative stress is a …
Alterations in stress granule dynamics driven by TDP-43 and FUS: a link to pathological inclusions in ALS?
A Aulas, C Vande Velde - Frontiers in cellular neuroscience, 2015 - frontiersin.org
Stress granules (SGs) are RNA-containing cytoplasmic foci formed in response to stress
exposure. Since their discovery in 1999, over 120 proteins have been described to be …
exposure. Since their discovery in 1999, over 120 proteins have been described to be …
Amyotrophic lateral sclerosis: mechanisms and therapeutics in the epigenomic era
X Paez-Colasante, C Figueroa-Romero… - Nature Reviews …, 2015 - nature.com
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease of the motor
neurons, which results in weakness and atrophy of voluntary skeletal muscles. Treatments …
neurons, which results in weakness and atrophy of voluntary skeletal muscles. Treatments …