[HTML][HTML] Emerging therapies and novel targets for TDP-43 proteinopathy in ALS/FTD

LR Hayes, P Kalab - Neurotherapeutics, 2022 - Elsevier
Nuclear clearance and cytoplasmic mislocalization of the essential RNA binding protein,
TDP-43, is a pathologic hallmark of amyotrophic lateral sclerosis, frontotemporal dementia …

Nuclear-import receptors as gatekeepers of pathological phase transitions in ALS/FTD

B Khalil, M Linsenmeier, CL Smith, J Shorter… - Molecular …, 2024 - Springer
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are fatal
neurodegenerative disorders on a disease spectrum that are characterized by the …

Monomerization of TDP-43 is a key determinant for inducing TDP-43 pathology in amyotrophic lateral sclerosis

K Oiwa, S Watanabe, K Onodera, Y Iguchi… - Science …, 2023 - science.org
The cytoplasmic aggregation of TAR DNA binding protein-43 (TDP-43), also known as TDP-
43 pathology, is the pathological hallmark of amyotrophic lateral sclerosis (ALS). However …

Loss of TDP‐43 oligomerization or RNA binding elicits distinct aggregation patterns

M Pérez‐Berlanga, VI Wiersma, A Zbinden… - The EMBO …, 2023 - embopress.org
Aggregation of the RNA‐binding protein TAR DNA‐binding protein 43 (TDP‐43) is the key
neuropathological feature of neurodegenerative diseases, including amyotrophic lateral …

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 …

RNA-mediated ribonucleoprotein assembly controls TDP-43 nuclear retention

PM Dos Passos, EH Hemamali, LD Mamede… - Plos …, 2024 - journals.plos.org
TDP-43 is an essential RNA-binding protein strongly implicated in the pathogenesis of
neurodegenerative disorders characterized by cytoplasmic aggregates and loss of nuclear …

RNA-binding deficient TDP-43 drives cognitive decline in a mouse model of TDP-43 proteinopathy

JC Necarsulmer, JM Simon, BA Evangelista, Y Chen… - Elife, 2023 - elifesciences.org
TDP-43 proteinopathies including frontotemporal lobar degeneration (FTLD) and
amyotrophic lateral sclerosis (ALS) are neurodegenerative disorders characterized by …

Expanding the TDP-43 proteinopathy pathway from neurons to muscle: physiological and pathophysiological functions

L Versluys, P Ervilha Pereira, N Schuermans… - Frontiers in …, 2022 - frontiersin.org
TAR DNA-binding protein 43, mostly referred to as TDP-43 (encoded by the TARDBP gene)
is strongly linked to the pathogenesis of amyotrophic lateral sclerosis (ALS) and …

Roles of RNA-binding proteins in neurological disorders, COVID-19, and cancer

DRA Sanya, C Cava, D Onésime - Human Cell, 2023 - Springer
RNA-binding proteins (RBPs) have emerged as important players in multiple biological
processes including transcription regulation, splicing, R-loop homeostasis, DNA …

The implications of physiological biomolecular condensates in amyotrophic lateral sclerosis

H Fakim, CV Velde - Seminars in Cell & Developmental Biology, 2024 - Elsevier
In recent years, there has been an emphasis on the role of phase-separated biomolecular
condensates, especially stress granules, in neurodegenerative diseases such as …