TARDBP mutation analysis in TDP-43 proteinopathies and deciphering the toxicity of mutant TDP-43
TF Gendron, R Rademakers… - Journal of Alzheimer's …, 2013 - content.iospress.com
The identification of TAR DNA-binding protein 43 (TDP-43) as the major disease protein in
amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitin …
amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitin …
Regulation of TDP-43 phosphorylation in aging and disease
Insoluble inclusions of phosphorylated TDP-43 occur in disease-affected neurons of most
patients with amyotrophic lateral sclerosis (ALS) and about half of patients with …
patients with amyotrophic lateral sclerosis (ALS) and about half of patients with …
Recapitulation of pathological TDP-43 features in immortalized lymphocytes from sporadic ALS patients
D Posa, L Martínez-González, F Bartolomé… - Molecular …, 2019 - Springer
Amyotrophic lateral sclerosis (ALS) is a fatal progressive neurodegenerative disorder of still
unknown etiology that results in loss of motoneurons, paralysis, and death, usually between …
unknown etiology that results in loss of motoneurons, paralysis, and death, usually between …
TDP-43: a novel neurodegenerative proteinopathy
MS Forman, JQ Trojanowski, VMY Lee - Current opinion in neurobiology, 2007 - Elsevier
Over the past decade, it has become clear that there is a significant overlap in the clinical
spectrum of frontotemporal lobar degeneration and amyotrophic lateral sclerosis (ALS). The …
spectrum of frontotemporal lobar degeneration and amyotrophic lateral sclerosis (ALS). The …
Neuron loss and degeneration in the progression of TDP-43 in frontotemporal lobar degeneration
Frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) is associated with
the accumulation of pathological neuronal and glial intracytoplasmic inclusions as well as …
the accumulation of pathological neuronal and glial intracytoplasmic inclusions as well as …
The molecular links between TDP‐43 dysfunction and neurodegeneration
E Buratti, FE Baralle - Advances in genetics, 2009 - Elsevier
TDP-43 nuclear protein is involved in several major neurodegenerative diseases that
include frontotemporal lobar degeneration with ubiquitin (FTLD-U) bodies and amyotrophic …
include frontotemporal lobar degeneration with ubiquitin (FTLD-U) bodies and amyotrophic …
[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 …
Distinct TDP-43 inclusion morphologies in frontotemporal lobar degeneration with and without amyotrophic lateral sclerosis
RH Tan, Y Yang, WS Kim, C Dobson-Stone… - Acta neuropathologica …, 2017 - Springer
The identification of the TAR DNA-binding protein 43 (TDP-43) as the ubiquitinated
cytoplasmic inclusions in frontotemporal lobar degeneration (FTLD) and amyotrophic lateral …
cytoplasmic inclusions in frontotemporal lobar degeneration (FTLD) and amyotrophic lateral …
Sense-encoded poly-GR dipeptide repeat proteins correlate to neurodegeneration and uniquely co-localize with TDP-43 in dendrites of repeat-expanded C9orf72 …
Hexanucleotide repeat expansions in C9orf72 are the most common genetic cause of
amyotrophic lateral sclerosis (C9 ALS). The main hypothesized pathogenic mechanisms are …
amyotrophic lateral sclerosis (C9 ALS). The main hypothesized pathogenic mechanisms are …
Molecular dissection of TDP-43 proteinopathies
M Hasegawa, T Nonaka, H Tsuji, A Tamaoka… - Journal of Molecular …, 2011 - Springer
TDP-43 has been identified as a major component of ubiquitin-positive tau-negative
cytoplasmic inclusions in frontotemporal lobar degeneration with ubiquitin-positive …
cytoplasmic inclusions in frontotemporal lobar degeneration with ubiquitin-positive …