[HTML][HTML] Synaptic dysfunction in ALS and FTD: anatomical and molecular changes provide insights into mechanisms of disease

PA Gelon, PA Dutchak, CF Sephton - Frontiers in Molecular …, 2022 - frontiersin.org
Synaptic loss is a pathological feature of all neurodegenerative diseases including
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). ALS is a disease of …

[HTML][HTML] Recent progress of the genetics of amyotrophic lateral sclerosis and challenges of gene therapy

H Wang, LP Guan, M Deng - Frontiers in neuroscience, 2023 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by the
degeneration of motor neurons in the brain and spinal cord. The causes of ALS are not fully …

Post-translational modifications on RNA-binding proteins: accelerators, brakes, or passengers in neurodegeneration?

EL Sternburg, LAG da Silva, D Dormann - Trends in biochemical sciences, 2022 - cell.com
RNA-binding proteins (RBPs) are critical players in RNA expression and metabolism, thus,
the proper regulation of this class of proteins is critical for cellular health. Regulation of RBPs …

[HTML][HTML] Boosting peripheral BDNF rescues impaired in vivo axonal transport in CMT2D mice

JN Sleigh, D Villarroel-Campos, S Surana… - JCI insight, 2023 - ncbi.nlm.nih.gov
Gain-of-function mutations in the housekeeping gene GARS1, which lead to the expression
of toxic versions of glycyl-tRNA synthetase (GlyRS), cause the selective motor and sensory …

[HTML][HTML] ALS-related FUS mutations alter axon growth in motoneurons and affect HuD/ELAVL4 and FMRP activity

MG Garone, N Birsa, M Rosito, F Salaris… - Communications …, 2021 - nature.com
Mutations in the RNA-binding protein (RBP) FUS have been genetically associated with the
motoneuron disease amyotrophic lateral sclerosis (ALS). Using both human induced …

[HTML][HTML] Phase separation and pathologic transitions of RNP condensates in neurons: implications for amyotrophic lateral sclerosis, frontotemporal dementia and other …

A Naskar, A Nayak, MR Salaikumaran… - Frontiers in Molecular …, 2023 - frontiersin.org
Liquid–liquid phase separation results in the formation of dynamic biomolecular
condensates, also known as membrane-less organelles, that allow for the assembly of …

[HTML][HTML] Coupling axonal mRNA transport and local translation to organelle maintenance and function

JNS Vargas, JN Sleigh, G Schiavo - Current Opinion in Cell Biology, 2022 - Elsevier
Neuronal homeostasis requires the transport of various organelles to distal compartments
and defects in this process lead to neurological disorders. Although several mechanisms for …

Lost in local translation: TDP-43 and FUS in axonal/neuromuscular junction maintenance and dysregulation in amyotrophic lateral sclerosis

D Piol, T Robberechts, S Da Cruz - Neuron, 2023 - cell.com
Key early features of amyotrophic lateral sclerosis (ALS) are denervation of neuromuscular
junctions and axonal degeneration. Motor neuron homeostasis relies on local translation …

[HTML][HTML] RNA granules in neuronal plasticity and disease

KE Bauer, BR de Queiroz, MA Kiebler, F Besse - Trends in Neurosciences, 2023 - cell.com
RNA granules are dynamic entities controlling the spatiotemporal distribution and translation
of RNA molecules. In neurons, a variety of RNA granules exist both in the soma and in …

[HTML][HTML] Translation dysregulation in neurodegenerative diseases: a focus on ALS

S Wang, S Sun - Molecular neurodegeneration, 2023 - Springer
RNA translation is tightly controlled in eukaryotic cells to regulate gene expression and
maintain proteome homeostasis. RNA binding proteins, translation factors, and cell …