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 …

Molecular Dissection of TDP-43 as a Leading Cause of ALS/FTLD

Y Tamaki, M Urushitani - International journal of molecular sciences, 2022 - mdpi.com
TAR DNA binding protein 43 (TDP-43) is a DNA/RNA binding protein involved in pivotal
cellular functions, especially in RNA metabolism. Hyperphosphorylated and ubiquitinated …

Pathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD–ALS spectrum disorders

J Janssens, C Van Broeckhoven - Human molecular genetics, 2013 - academic.oup.com
Aggregation of misfolded TAR DNA-binding protein 43 (TDP-43) is a striking hallmark of
neurodegenerative processes that are observed in several neurological disorders, and in …

Low molecular weight species of TDP-43 generated by abnormal splicing form inclusions in amyotrophic lateral sclerosis and result in motor neuron death

S Xiao, T Sanelli, H Chiang, Y Sun, A Chakrabartty… - Acta …, 2015 - Springer
The presence of lower molecular weight species comprising the C-terminal region of TAR
DNA-binding protein 43 (TDP-43) is a characteristic of TDP-43 proteinopathy in amyotrophic …

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 …

Novel Mutations in TARDBP (TDP-43) in Patients with Familial Amyotrophic Lateral Sclerosis

NJ Rutherford, YJ Zhang, M Baker, JM Gass… - PLoS …, 2008 - journals.plos.org
The TAR DNA-binding protein 43 (TDP-43) has been identified as the major disease protein
in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitin …

The role of TDP-43 mislocalization in amyotrophic lateral sclerosis

TR Suk, MWC Rousseaux - Molecular neurodegeneration, 2020 - Springer
Since its discovery as a primary component in cytoplasmic aggregates in post-mortem tissue
of patients with Amyotrophic Lateral Sclerosis (ALS), TAR DNA Binding Protein 43 kDa (TDP …

TAR DNA-binding protein 43 in neurodegenerative disease

AS Chen-Plotkin, VMY Lee… - Nature Reviews …, 2010 - nature.com
In 2006, TAR DNA-binding protein 43 (TDP-43), a highly conserved nuclear protein, was
identified as the major disease protein in amyotrophic lateral sclerosis (ALS) and in the most …

Overexpression of ALS-associated p. M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice

J Janssens, H Wils, G Kleinberger, G Joris, I Cuijt… - Molecular …, 2013 - Springer
Mutations in TAR DNA-binding protein 43 (TDP-43) are associated with familial forms of
amyotrophic lateral sclerosis (ALS), while wild-type TDP-43 is a pathological hallmark of …

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 …