Pathomechanisms of TDP‐43 in neurodegeneration
Neurodegeneration, a term that refers to the progressive loss of structure and function of
neurons, is a feature of many neurodegenerative diseases such as amyotrophic lateral …
neurons, is a feature of many neurodegenerative diseases such as amyotrophic lateral …
[HTML][HTML] The role of hnRNPs in frontotemporal dementia and amyotrophic lateral sclerosis
Dysregulated RNA metabolism is emerging as a crucially important mechanism
underpinning the pathogenesis of frontotemporal dementia (FTD) and the clinically …
underpinning the pathogenesis of frontotemporal dementia (FTD) and the clinically …
[HTML][HTML] TDP-43 loss and ALS-risk SNPs drive mis-splicing and depletion of UNC13A
Variants of UNC13A, a critical gene for synapse function, increase the risk of amyotrophic
lateral sclerosis and frontotemporal dementia,–, two related neurodegenerative diseases …
lateral sclerosis and frontotemporal dementia,–, two related neurodegenerative diseases …
Mis-spliced transcripts generate de novo proteins in TDP-43–related ALS/FTD
Functional loss of TDP-43, an RNA binding protein genetically and pathologically linked to
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), leads to the …
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), leads to the …
Premature polyadenylation-mediated loss of stathmin-2 is a hallmark of TDP-43-dependent neurodegeneration
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are associated with
loss of nuclear transactive response DNA-binding protein 43 (TDP-43). Here we identify that …
loss of nuclear transactive response DNA-binding protein 43 (TDP-43). Here we identify that …
ALS-implicated protein TDP-43 sustains levels of STMN2, a mediator of motor neuron growth and repair
JR Klim, LA Williams, F Limone… - Nature …, 2019 - nature.com
The findings that amyotrophic lateral sclerosis (ALS) patients almost universally display
pathological mislocalization of the RNA-binding protein TDP-43 and that mutations in its …
pathological mislocalization of the RNA-binding protein TDP-43 and that mutations in its …
[HTML][HTML] Truncated stathmin-2 is a marker of TDP-43 pathology in frontotemporal dementia
M Prudencio, J Humphrey, S Pickles… - The Journal of …, 2020 - Am Soc Clin Investig
No treatment for frontotemporal dementia (FTD), the second most common type of early-
onset dementia, is available, but therapeutics are being investigated to target the 2 main …
onset dementia, is available, but therapeutics are being investigated to target the 2 main …
[HTML][HTML] Functional and dynamic polymerization of the ALS-linked protein TDP-43 antagonizes its pathologic aggregation
TDP-43 is a primarily nuclear RNA-binding protein, whose abnormal phosphorylation and
cytoplasmic aggregation characterizes affected neurons in patients with amyotrophic lateral …
cytoplasmic aggregation characterizes affected neurons in patients with amyotrophic lateral …
[HTML][HTML] TDP-43-regulated cryptic RNAs accumulate in Alzheimer's disease brains
V Estades Ayuso, S Pickles, T Todd, M Yue… - Molecular …, 2023 - Springer
Abstract Background Inclusions of TAR DNA-binding protein 43 kDa (TDP-43) has been
designated limbic-predominant, age-related TDP-43 encephalopathy (LATE), with or without …
designated limbic-predominant, age-related TDP-43 encephalopathy (LATE), with or without …
[HTML][HTML] Cryptic exon detection and transcriptomic changes revealed in single-nuclei RNA sequencing of C9ORF72 patients spanning the ALS-FTD spectrum
The C9ORF72-linked diseases amyotrophic lateral sclerosis (ALS) and frontotemporal
dementia (FTD) are characterized by the nuclear depletion and cytoplasmic accumulation of …
dementia (FTD) are characterized by the nuclear depletion and cytoplasmic accumulation of …