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 …
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 …
Structural insights into TDP-43 and effects of post-translational modifications
Transactive response DNA binding protein (TDP-43) is a key player in neurodegenerative
diseases. In this review, we have gathered and presented structural information on the …
diseases. In this review, we have gathered and presented structural information on the …
RNA-binding proteins in neurodegeneration: mechanisms in aggregate
Neurodegeneration is a leading cause of death in the developed world and a natural, albeit
unfortunate, consequence of longer-lived populations. Despite great demand for therapeutic …
unfortunate, consequence of longer-lived populations. Despite great demand for therapeutic …
The role of VCP mutations in the Spectrum of amyotrophic lateral sclerosis—frontotemporal dementia
E Scarian, G Fiamingo, L Diamanti, I Palmieri… - Frontiers in …, 2022 - frontiersin.org
Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Dementia (FTD) are two
neurological diseases which, respectively, and primarily affect motor neurons and …
neurological diseases which, respectively, and primarily affect motor neurons and …
Solid-state NMR reveals the structural transformation of the TDP-43 amyloidogenic region upon fibrillation
XF Zhuo, J Wang, J Zhang, LL Jiang… - Journal of the …, 2020 - ACS Publications
TDP-43 is a primary pathological hallmark protein of amyotrophic lateral sclerosis and
frontotemporal lobar degeneration, which may exist in the form of amyloid inclusions in the …
frontotemporal lobar degeneration, which may exist in the form of amyloid inclusions in the …
Point mutations in the N-terminal domain of transactive response DNA-binding protein 43 kDa (TDP-43) compromise its stability, dimerization, and functions
M Mompeán, V Romano, D Pantoja-Uceda… - Journal of Biological …, 2017 - ASBMB
Transactive response DNA-binding protein 43 (TDP-43) performs multiple tasks in mRNA
processing, transport, and translational regulation, but it also forms aggregates implicated in …
processing, transport, and translational regulation, but it also forms aggregates implicated in …
Zinc binding to RNA recognition motif of TDP-43 induces the formation of amyloid-like aggregates
Aggregation of TDP-43 (transactive response DNA binding protein 43 kDa) is a hallmark of
certain forms of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration …
certain forms of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration …
[HTML][HTML] Intercellular transmission of pathogenic proteins in ALS: Exploring the pathogenic wave
FJ Arnold, AD Nguyen, RS Bedlack, CL Bennett… - Neurobiology of …, 2023 - Elsevier
In patients with amyotrophic lateral sclerosis (ALS), disease symptoms and pathology
typically spread in a predictable spatiotemporal pattern beginning at a focal site of onset and …
typically spread in a predictable spatiotemporal pattern beginning at a focal site of onset and …
The RNA-recognition motifs of TAR DNA-binding protein 43 may play a role in the aberrant self-assembly of the protein
The TAR DNA-binding protein 43 (TDP-43) is a nucleic acid-binding protein implicated in
gene regulation and RNA processing and shuffling. It is a ribonuclear protein that carries out …
gene regulation and RNA processing and shuffling. It is a ribonuclear protein that carries out …