[HTML][HTML] The role of TDP-43 mislocalization in amyotrophic lateral sclerosis
TR Suk, MWC Rousseaux - Molecular neurodegeneration, 2020 - Springer
Since its discovery as a primary component in cytoplasmic aggregates in post-mortem tissue
of patients with Amyotrophic Lateral Sclerosis (ALS), TAR DNA Binding Protein 43 kDa (TDP …
of patients with Amyotrophic Lateral Sclerosis (ALS), TAR DNA Binding Protein 43 kDa (TDP …
Triad of TDP43 control in neurodegeneration: autoregulation, localization and aggregation
P Tziortzouda, L Van Den Bosch, F Hirth - Nature Reviews …, 2021 - nature.com
Cytoplasmic aggregation of TAR DNA-binding protein 43 (TDP43; also known as TARDBP
or TDP-43) is a key pathological feature of several neurodegenerative diseases, including …
or TDP-43) is a key pathological feature of several neurodegenerative diseases, including …
RNA buffers the phase separation behavior of prion-like RNA binding proteins
Prion-like RNA binding proteins (RBPs) such as TDP43 and FUS are largely soluble in the
nucleus but form solid pathological aggregates when mislocalized to the cytoplasm. What …
nucleus but form solid pathological aggregates when mislocalized to the cytoplasm. What …
The C9orf72 repeat expansion disrupts nucleocytoplasmic transport
The hexanucleotide repeat expansion (HRE) GGGGCC (G4C2) in C9orf72 is the most
common cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) …
common cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) …
[HTML][HTML] Lysosome dysfunction as a cause of neurodegenerative diseases: Lessons from frontotemporal dementia and amyotrophic lateral sclerosis
Frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) are fatal
neurodegenerative disorders that are thought to exist on a clinical and pathological …
neurodegenerative disorders that are thought to exist on a clinical and pathological …
[HTML][HTML] Axonal transport of TDP-43 mRNA granules is impaired by ALS-causing mutations
NH Alami, RB Smith, MA Carrasco, LA Williams… - Neuron, 2014 - cell.com
The RNA-binding protein TDP-43 regulates RNA metabolism at multiple levels, including
transcription, RNA splicing, and mRNA stability. TDP-43 is a major component of the …
transcription, RNA splicing, and mRNA stability. TDP-43 is a major component of the …
Drosophila as an In Vivo Model for Human Neurodegenerative Disease
With the increase in the ageing population, neurodegenerative disease is devastating to
families and poses a huge burden on society. The brain and spinal cord are extraordinarily …
families and poses a huge burden on society. The brain and spinal cord are extraordinarily …
[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] RNA dysregulation in amyotrophic lateral sclerosis
Z Butti, SA Patten - Frontiers in genetics, 2019 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is the most common adult-onset motor neuron disease
and is characterized by the degeneration of upper and lower motor neurons. It has become …
and is characterized by the degeneration of upper and lower motor neurons. It has become …
[HTML][HTML] TDP-43 depletion in microglia promotes amyloid clearance but also induces synapse loss
Microglia coordinate various functions in the central nervous system ranging from removing
synaptic connections, to maintaining brain homeostasis by monitoring neuronal function …
synaptic connections, to maintaining brain homeostasis by monitoring neuronal function …