Concomitant TAR-DNA-binding protein 43 pathology is present in Alzheimer disease and corticobasal degeneration but not in other tauopathies

K Uryu, H Nakashima-Yasuda… - … of Neuropathology & …, 2008 - academic.oup.com
Pathologic TAR-DNA-binding protein 43 (TDP-43) is a disease protein in frontotemporal
lobar degeneration with ubiquitin-positive inclusions (FTLD-U) and amyotrophic lateral …

FUS mutations in frontotemporal lobar degeneration with amyotrophic lateral sclerosis

O Broustal, A Camuzat, L Guillot-Noël… - Journal of …, 2010 - journals.sagepub.com
Rapid advances were made in the knowledge of amyotrophic lateral sclerosis (ALS) with the
recent identification of TARDBP and FUS mutations in familial ALS. More recently, FUS …

Nuclear import impairment causes cytoplasmic trans-activation response DNA-binding protein accumulation and is associated with frontotemporal lobar degeneration

AL Nishimura, V Župunski, C Troakes, C Kathe… - Brain, 2010 - academic.oup.com
Trans-activation response DNA-binding protein (TDP-43) accumulation is the major
component of ubiquitinated protein inclusions found in patients with amyotrophic lateral …

Sporadic amyotrophic lateral sclerosis: two pathological patterns shown by analysis of distribution of TDP-43-immunoreactive neuronal and glial cytoplasmic …

Y Nishihira, CF Tan, O Onodera, Y Toyoshima… - Acta …, 2008 - Springer
A nuclear protein, 43-kDa TAR DNA-binding protein (TDP-43), was recently identified as a
component of the ubiquitinated inclusions (UIs) in frontotemporal lobar degeneration (FTLD …

Quantification of the Relative Contributions of Loss-of-function and Gain-of-function Mechanisms in TAR DNA-binding Protein 43 (TDP-43) Proteinopathies

R Cascella, C Capitini, G Fani, CM Dobson… - Journal of Biological …, 2016 - ASBMB
Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitin
positive inclusions (FTLD-U) are two clinically distinct neurodegenerative conditions sharing …

TDP-43 forms amyloid filaments with a distinct fold in type A FTLD-TDP

D Arseni, R Chen, AG Murzin, SY Peak-Chew… - Nature, 2023 - nature.com
The abnormal assembly of TAR DNA-binding protein 43 (TDP-43) in neuronal and glial cells
characterizes nearly all cases of amyotrophic lateral sclerosis (ALS) and around half of …

TDP‐43: the relationship between protein aggregation and neurodegeneration in amyotrophic lateral sclerosis and frontotemporal lobar degeneration

RH Baloh - The FEBS journal, 2011 - Wiley Online Library
Accumulations of aggregated proteins are a key feature of the pathology of all of the major
neurodegenerative diseases. Amyotrophic lateral sclerosis (ALS) was brought into this fold …

Disease causing mutants of TDP-43 nucleic acid binding domains are resistant to aggregation and have increased stability and half-life

JA Austin, GSA Wright, S Watanabe… - Proceedings of the …, 2014 - National Acad Sciences
Over the last two decades many secrets of the age-related human neural proteinopathies
have been revealed. A common feature of these diseases is abnormal, and possibly …

Enrichment of C-terminal fragments in TAR DNA-binding protein-43 cytoplasmic inclusions in brain but not in spinal cord of frontotemporal lobar degeneration and …

LM Igaz, LK Kwong, Y Xu, AC Truax, K Uryu… - The American journal of …, 2008 - Elsevier
TAR DNA-binding protein (TDP-43) has been recently described as a major pathological
protein in both frontotemporal dementia with ubiquitin-positive inclusions (FTLD-U) and …

The dual functions of the extreme N-terminus of TDP-43 in regulating its biological activity and inclusion formation

YJ Zhang, T Caulfield, YF Xu… - Human molecular …, 2013 - academic.oup.com
Abstract TAR DNA-binding protein-43 (TDP-43) is the principal component of ubiquitinated
inclusions in amyotrophic lateral sclerosis (ALS) and the most common pathological subtype …