The nexus between RNA-binding proteins and their effectors
RNA-binding proteins (RBPs) regulate essentially every event in the lifetime of an RNA
molecule, from its production to its destruction. Whereas much has been learned about RNA …
molecule, from its production to its destruction. Whereas much has been learned about RNA …
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 …
[PDF][PDF] Lost in transportation: nucleocytoplasmic transport defects in ALS and other neurodegenerative diseases
HJ Kim, JP Taylor - Neuron, 2017 - cell.com
Amyotrophic lateral sclerosis (ALS) is a progressive, fatal neurodegenerative disease
characterized by degeneration of upper and lower motor neurons in the brain and spinal …
characterized by degeneration of upper and lower motor neurons in the brain and spinal …
[PDF][PDF] Disruption of RNA metabolism in neurological diseases and emerging therapeutic interventions
RNA binding proteins are critical to the maintenance of the transcriptome via controlled
regulation of RNA processing and transport. Alterations of these proteins impact multiple …
regulation of RNA processing and transport. Alterations of these proteins impact multiple …
ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function
A Sharma, AK Lyashchenko, L Lu… - Nature …, 2016 - nature.com
Mutations in FUS cause amyotrophic lateral sclerosis (ALS), including some of the most
aggressive, juvenile-onset forms of the disease. FUS loss-of-function and toxic gain-of …
aggressive, juvenile-onset forms of the disease. FUS loss-of-function and toxic gain-of …
Patient-derived frontotemporal lobar degeneration brain extracts induce formation and spreading of TDP-43 pathology in vivo
S Porta, Y Xu, CR Restrepo, LK Kwong, B Zhang… - Nature …, 2018 - nature.com
The stereotypical distribution of TAR DNA-binding 43 protein (TDP-43) aggregates in
frontotemporal lobar degeneration (FTLD-TDP) suggests that pathological TDP-43 spreads …
frontotemporal lobar degeneration (FTLD-TDP) suggests that pathological TDP-43 spreads …
[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 …
The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease
Prions are self-templating protein conformers that are naturally transmitted between
individuals and promote phenotypic change. In yeast, prion-encoded phenotypes can be …
individuals and promote phenotypic change. In yeast, prion-encoded phenotypes can be …
TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia
IRA Mackenzie, R Rademakers… - The Lancet Neurology, 2010 - thelancet.com
Abnormal intracellular protein aggregates comprise a key characteristic in most
neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and …
neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and …
Interactions between ALS-linked FUS and nucleoporins are associated with defects in the nucleocytoplasmic transport pathway
YC Lin, MS Kumar, N Ramesh, EN Anderson… - Nature …, 2021 - nature.com
Nucleocytoplasmic transport (NCT) decline occurs with aging and neurodegeneration. Here,
we investigated the NCT pathway in models of amyotrophic lateral sclerosis–fused in …
we investigated the NCT pathway in models of amyotrophic lateral sclerosis–fused in …