The role of TDP-43 propagation in neurodegenerative diseases: integrating insights from clinical and experimental studies

M Jo, S Lee, YM Jeon, S Kim, Y Kwon… - Experimental & molecular …, 2020 - nature.com
Abstract TAR DNA-binding protein 43 (TDP-43) is a highly conserved nuclear RNA/DNA-
binding protein involved in the regulation of RNA processing. The accumulation of TDP-43 …

TDP-43 loss of cellular function through aggregation requires additional structural determinants beyond its C-terminal Q/N prion-like domain

M Budini, V Romano, Z Quadri, E Buratti… - Human molecular …, 2015 - academic.oup.com
TDP-43 aggregates are the neurohistological landmark of diseases like amyotrophic lateral
sclerosis and frontotemporal dementia. Their role in the pathogenesis of these conditions is …

TDP-43 pathology: from noxious assembly to therapeutic removal

SS Keating, R San Gil, MEV Swanson, EL Scotter… - Progress in …, 2022 - Elsevier
Our understanding of amyotrophic lateral sclerosis and frontotemporal dementia has
advanced dramatically since the discovery of cytoplasmic TAR DNA-binding protein 43 (TDP …

Prion-like properties of pathological TDP-43 aggregates from diseased brains

T Nonaka, M Masuda-Suzukake, T Arai, Y Hasegawa… - Cell reports, 2013 - cell.com
TDP-43 is the major component protein of ubiquitin-positive inclusions in brains of patients
with frontotemporal lobar degeneration (FTLD-TDP) or amyotrophic lateral sclerosis (ALS) …

TDP-43: gumming up neurons through protein–protein and protein–RNA interactions

E Buratti, FE Baralle - Trends in biochemical sciences, 2012 - cell.com
Since the discovery that 43 kDa TAR DNA binding protein (TDP-43) is involved in
neurodegeneration, studies of this protein have focused on the global effects of TDP-43 …

Cellular model of TAR DNA-binding protein 43 (TDP-43) aggregation based on its C-terminal Gln/Asn-rich region

M Budini, E Buratti, C Stuani, C Guarnaccia… - Journal of Biological …, 2012 - ASBMB
TDP-43 is one of the major components of the neuronal and glial inclusions observed in
several neurodegenerative diseases such as amyotrophic lateral sclerosis and …

TDP-43 proteinopathies: a new wave of neurodegenerative diseases

EMJ De Boer, VK Orie, T Williams, MR Baker… - Journal of Neurology …, 2021 - jnnp.bmj.com
Inclusions of pathogenic deposits containing TAR DNA-binding protein 43 (TDP-43) are
evident in the brain and spinal cord of patients that present across a spectrum of …

A yeast TDP-43 proteinopathy model: Exploring the molecular determinants of TDP-43 aggregation and cellular toxicity

BS Johnson, JM McCaffery… - Proceedings of the …, 2008 - National Acad Sciences
Protein misfolding is intimately associated with devastating human neurodegenerative
diseases, including Alzheimer's, Huntington's, and Parkinson's. Although disparate in their …

TDP-43 pathology in Alzheimer's disease

A Meneses, S Koga, J O'Leary, DW Dickson… - Molecular …, 2021 - Springer
Transactive response DNA binding protein of 43 kDa (TDP-43) is an intranuclear protein
encoded by the TARDBP gene that is involved in RNA splicing, trafficking, stabilization, and …

TDP-43: an emerging new player in neurodegenerative diseases

IF Wang, LS Wu, CKJ Shen - Trends in molecular medicine, 2008 - cell.com
Until a couple of years ago, TAR-DNA-binding protein-43 (TDP-43) was a relatively
unknown nuclear protein implicated in transcriptional repression and splicing. Since 2006 …