ALS genetics, mechanisms, and therapeutics: where are we now?
The scientific landscape surrounding amyotrophic lateral sclerosis (ALS) continues to shift
as the number of genes associated with the disease risk and pathogenesis, and the cellular …
as the number of genes associated with the disease risk and pathogenesis, and the cellular …
Nuclear speckles: molecular organization, biological function and role in disease
L Galganski, MO Urbanek… - Nucleic acids …, 2017 - academic.oup.com
The nucleoplasm is not homogenous; it consists of many types of nuclear bodies, also
known as nuclear domains or nuclear subcompartments. These self-organizing structures …
known as nuclear domains or nuclear subcompartments. These self-organizing structures …
Phosphorylation of the FUS low‐complexity domain disrupts phase separation, aggregation, and toxicity
Neuronal inclusions of aggregated RNA‐binding protein fused in sarcoma (FUS) are
hallmarks of ALS and frontotemporal dementia subtypes. Intriguingly, FUS's nearly …
hallmarks of ALS and frontotemporal dementia subtypes. Intriguingly, FUS's nearly …
Oxidative stress in amyotrophic lateral sclerosis: pathophysiology and opportunities for pharmacological intervention
T Cunha-Oliveira, L Montezinho… - Oxidative medicine …, 2020 - Wiley Online Library
Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease or Charcot
disease, is a fatal neurodegenerative disease that affects motor neurons (MNs) and leads to …
disease, is a fatal neurodegenerative disease that affects motor neurons (MNs) and leads to …
Liquid-liquid phase separation of TDP-43 and FUS in physiology and pathology of neurodegenerative diseases
JL Carey, L Guo - Frontiers in molecular biosciences, 2022 - frontiersin.org
Liquid-liquid phase separation of RNA-binding proteins mediates the formation of numerous
membraneless organelles with essential cellular function. However, aberrant phase …
membraneless organelles with essential cellular function. However, aberrant phase …
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] Antisense Oligonucleotides for the Study and Treatment of ALS
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by
motor neuron loss. ALS is now associated with mutations in numerous genes, many of which …
motor neuron loss. ALS is now associated with mutations in numerous genes, many of which …
Neuropathology of amyotrophic lateral sclerosis and its variants
S Saberi, JE Stauffer, DJ Schulte… - Neurologic …, 2015 - neurologic.theclinics.com
The first case reports of amyotrophic lateral sclerosis (ALS) date back to Charles Bell in
1824. 1 Although a variety of other clinical descriptions followed throughout the 1850s, 2–4 …
1824. 1 Although a variety of other clinical descriptions followed throughout the 1850s, 2–4 …
Genetic mutations in RNA-binding proteins and their roles in ALS
K Kapeli, FJ Martinez, GW Yeo - Human genetics, 2017 - Springer
Mutations in genes that encode RNA-binding proteins (RBPs) have emerged as critical
determinants of neurological diseases, especially motor neuron disorders such as …
determinants of neurological diseases, especially motor neuron disorders such as …
Molecular and cellular mechanisms affected in ALS
L Le Gall, E Anakor, O Connolly… - Journal of personalized …, 2020 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is a terminal late-onset condition characterized by the
loss of upper and lower motor neurons. Mutations in more than 30 genes are associated to …
loss of upper and lower motor neurons. Mutations in more than 30 genes are associated to …