Rethinking als: The fus about tdp-43

C Lagier-Tourenne, DW Cleveland - Cell, 2009 - cell.com
Mutations in TDP-43, a DNA/RNA-binding protein, cause an inherited form of the
neurodegenerative disease amyotrophic lateral sclerosis (ALS). Two recent studies …

Understanding the role of TDP-43 and FUS/TLS in ALS and beyond

S Da Cruz, DW Cleveland - Current opinion in neurobiology, 2011 - Elsevier
Dominant mutations in two DNA/RNA binding proteins, TDP-43 and FUS/TLS, are causes of
inherited Amyotrophic Lateral Sclerosis (ALS). TDP-43 and FUS/TLS have striking structural …

TDP-43/FUS in motor neuron disease: Complexity and challenges

EN Guerrero, H Wang, J Mitra, PM Hegde… - Progress in …, 2016 - Elsevier
Amyotrophic lateral sclerosis (ALS), a common motor neuron disease affecting two per
100,000 people worldwide, encompasses at least five distinct pathological subtypes …

TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration

C Lagier-Tourenne, M Polymenidou… - Human molecular …, 2010 - academic.oup.com
Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are
neurodegenerative diseases with clinical and pathological overlap. Landmark discoveries of …

Rodent models of TDP-43: recent advances

W Tsao, YH Jeong, S Lin, J Ling, DL Price, PM Chiang… - Brain research, 2012 - Elsevier
Recently, missense mutations in the gene TARDBP encoding TDP-43 have been linked to
familial ALS. The discovery of genes encoding these RNA binding proteins, such as TDP-43 …

[HTML][HTML] The ALS/FTLD-related RNA-binding proteins TDP-43 and FUS have common downstream RNA targets in cortical neurons

D Honda, S Ishigaki, Y Iguchi, Y Fujioka, T Udagawa… - FEBS open bio, 2014 - Elsevier
TDP-43 and FUS are linked to amyotrophic lateral sclerosis (ALS) and frontotemporal lobar
degeneration (FTLD), and loss of function of either protein contributes to these …

Amyotrophic lateral sclerosis-associated proteins TDP-43 and FUS/TLS function in a common biochemical complex to co-regulate HDAC6 mRNA

SH Kim, NP Shanware, MJ Bowler… - Journal of Biological …, 2010 - ASBMB
Amyotrophic lateral sclerosis (ALS) is an incurable neurodegenerative disease that
preferentially targets motor neurons. It was recently found that dominant mutations in two …

A robust TDP-43 knock-in mouse model of ALS

SL Huang, LS Wu, M Lee, CW Chang… - Acta neuropathologica …, 2020 - Springer
Amyotrophic lateral sclerosis (ALS) is a fatal, adult-onset degenerative disorder of motor
neurons. The diseased spinal cord motor neurons of more than 95% of amyotrophic lateral …

TDP-43-mediated neurodegeneration: towards a loss-of-function hypothesis?

LV Broeck, P Callaerts, B Dermaut - Trends in molecular medicine, 2014 - cell.com
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are clinically distinct
fatal neurodegenerative disorders. Increasing molecular evidence indicates that both …

Molecular mechanisms of TDP-43 misfolding and pathology in amyotrophic lateral sclerosis

A Prasad, V Bharathi, V Sivalingam… - Frontiers in molecular …, 2019 - frontiersin.org
TAR DNA binding protein 43 (TDP-43) is a versatile RNA/DNA binding protein involved in
RNA-related metabolism. Hyper-phosphorylated and ubiquitinated TDP-43 deposits act as …