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 …

[HTML][HTML] The role of amyloid oligomers in neurodegenerative pathologies

C Wells, S Brennan, M Keon, L Ooi - International Journal of Biological …, 2021 - Elsevier
Many neurodegenerative diseases are rooted in the activities of amyloid-like proteins which
possess conformations that spread to healthy proteins. These include Alzheimer's disease …

TDP-43 α-helical structure tunes liquid–liquid phase separation and function

AE Conicella, GL Dignon, GH Zerze… - Proceedings of the …, 2020 - National Acad Sciences
Liquid–liquid phase separation (LLPS) is involved in the formation of membraneless
organelles (MLOs) associated with RNA processing. The RNA-binding protein TDP-43 is …

The role of liquid–liquid phase separation in aggregation of the TDP-43 low-complexity domain

WM Babinchak, R Haider, BK Dumm, P Sarkar… - Journal of Biological …, 2019 - ASBMB
Pathological aggregation of the transactive response DNA-binding protein of 43 kDa (TDP-
43) is associated with several neurodegenerative disorders, including ALS, frontotemporal …

Structural insights into TDP-43 and effects of post-translational modifications

L François-Moutal, S Perez-Miller, DD Scott… - Frontiers in molecular …, 2019 - frontiersin.org
Transactive response DNA binding protein (TDP-43) is a key player in neurodegenerative
diseases. In this review, we have gathered and presented structural information on the …

The physical forces mediating self-association and phase-separation in the C-terminal domain of TDP-43

HR Li, TC Chen, CL Hsiao, L Shi, CY Chou… - Biochimica et Biophysica …, 2018 - Elsevier
The TAR DNA-binding protein of 43 kDa (TDP-43) has been identified as the main
component of amyotrophic lateral sclerosis (ALS) cytoplasmic inclusions. The link between …

Prion-like C-terminal domain of TDP-43 and α-Synuclein interact synergistically to generate neurotoxic hybrid fibrils

S Dhakal, CE Wyant, HE George, SE Morgan… - Journal of molecular …, 2021 - Elsevier
Aberrant aggregation and amyloid formation of tar DNA binding protein (TDP-43) and α-
synuclein (αS) underlie frontotemporal dementia (FTD) and Parkinson's disease (PD) …

[HTML][HTML] Intercellular transmission of pathogenic proteins in ALS: Exploring the pathogenic wave

FJ Arnold, AD Nguyen, RS Bedlack, CL Bennett… - Neurobiology of …, 2023 - Elsevier
In patients with amyotrophic lateral sclerosis (ALS), disease symptoms and pathology
typically spread in a predictable spatiotemporal pattern beginning at a focal site of onset and …

Molecular, functional, and pathological aspects of TDP-43 fragmentation

D Chhangani, A Martín-Peña, DE Rincon-Limas - IScience, 2021 - cell.com
Transactive response DNA binding protein 43 (TDP-43) is a DNA/RNA binding protein
involved in transcriptional regulation and RNA processing. It is linked to sporadic and …

Parkin beyond Parkinson's Disease—A Functional Meaning of Parkin Downregulation in TDP-43 Proteinopathies

K Gaweda-Walerych, EJ Sitek, E Narożańska, E Buratti - Cells, 2021 - mdpi.com
Parkin and PINK1 are key regulators of mitophagy, an autophagic pathway for selective
elimination of dysfunctional mitochondria. To this date, parkin depletion has been …