TDP-43 proteinopathy in frontotemporal lobar degeneration and amyotrophic lateral sclerosis: protein misfolding diseases without amyloidosis

M Neumann, LK Kwong, DM Sampathu… - Archives of …, 2007 - jamanetwork.com
Herein, we review advances in understanding a group of disorders collectively known as
TAR-DNA binding protein 43 (TDP-43) proteinopathies since the report that TDP-43 is the …

TDP-43 proteinopathies: neurodegenerative protein misfolding diseases without amyloidosis

LK Kwong, K Uryu, JQ Trojanowski, VMY Lee - Neurosignals, 2007 - karger.com
In this review, we summarize recent advances in understanding frontotemporal lobar
degeneration (FTLD), amyotrophic lateral sclerosis (ALS) and related neurodegenerative …

TDP-43 proteinopathy: the neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron disease

LK Kwong, M Neumann, DM Sampathu, VMY Lee… - Acta …, 2007 - Springer
The rapid confirmation of the initial report by Neumann et al.(Science 314: 130–133, 2006)
that transactive response (TAR)-DNA-binding protein 43 (TDP-43) is the major disease …

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 …

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 …

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 …

Molecular neuropathology of TDP-43 proteinopathies

M Neumann - International journal of molecular sciences, 2009 - mdpi.com
The identification of TDP-43 as the major component of the pathologic inclusions in most
forms of sporadic and familial frontotemporal lobar degeneration with ubiquitin-positive …

Truncation and pathogenic mutations facilitate the formation of intracellular aggregates of TDP-43

T Nonaka, F Kametani, T Arai, H Akiyama… - Human molecular …, 2009 - academic.oup.com
TAR DNA binding protein of 43 kDa (TDP-43) is a major component of the ubiquitin-positive
inclusions found in the brain of patients with frontotemporal lobar degeneration (FTLD-U) …

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 …

Amyotrophic lateral sclerosis, frontotemporal dementia and beyond: the TDP-43 diseases

F Geser, M Martinez-Lage, LK Kwong, VMY Lee… - Journal of …, 2009 - Springer
Ever since the significance of pathological 43-kDa transactivating responsive sequence
DNA-binding protein (TDP-43) for human disease has been recognized in amyotrophic …