[HTML][HTML] 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 …

Triad of TDP43 control in neurodegeneration: autoregulation, localization and aggregation

P Tziortzouda, L Van Den Bosch, F Hirth - Nature Reviews …, 2021 - nature.com
Cytoplasmic aggregation of TAR DNA-binding protein 43 (TDP43; also known as TARDBP
or TDP-43) is a key pathological feature of several neurodegenerative diseases, including …

[HTML][HTML] Practical considerations for choosing a mouse model of Alzheimer's disease

JL Jankowsky, H Zheng - Molecular neurodegeneration, 2017 - Springer
Alzheimer's disease (AD) is behaviorally identified by progressive memory impairment and
pathologically characterized by the triad of β-amyloid plaques, neurofibrillary tangles, and …

[HTML][HTML] 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 …

[HTML][HTML] Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis

SC Ling, M Polymenidou, DW Cleveland - Neuron, 2013 - cell.com
Breakthrough discoveries identifying common genetic causes for amyotrophic lateral
sclerosis (ALS) and frontotemporal dementia (FTD) have transformed our view of these …

Autophagy and ALS: mechanistic insights and therapeutic implications

JP Chua, H De Calbiac, E Kabashi, SJ Barmada - Autophagy, 2022 - Taylor & Francis
Mechanisms of protein homeostasis are crucial for overseeing the clearance of misfolded
and toxic proteins over the lifetime of an organism, thereby ensuring the health of neurons …

[HTML][HTML] Human endogenous retroviruses in neurological diseases

P Küry, A Nath, A Créange, A Dolei, P Marche… - Trends in molecular …, 2018 - cell.com
The causes of multiple sclerosis and amyotrophic lateral sclerosis have long remained
elusive. A new category of pathogenic components, normally dormant within human …

[HTML][HTML] Patient-derived frontotemporal lobar degeneration brain extracts induce formation and spreading of TDP-43 pathology in vivo

S Porta, Y Xu, CR Restrepo, LK Kwong, B Zhang… - Nature …, 2018 - nature.com
The stereotypical distribution of TAR DNA-binding 43 protein (TDP-43) aggregates in
frontotemporal lobar degeneration (FTLD-TDP) suggests that pathological TDP-43 spreads …

[HTML][HTML] Protein aggregation in amyotrophic lateral sclerosis

AM Blokhuis, EJN Groen, M Koppers… - Acta …, 2013 - Springer
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the
aggregation of ubiquitinated proteins in affected motor neurons. Recent studies have …

[HTML][HTML] Altered ribostasis: RNA-protein granules in degenerative disorders

M Ramaswami, JP Taylor, R Parker - Cell, 2013 - cell.com
The molecular processes that contribute to degenerative diseases are not well understood.
Recent observations suggest that some degenerative diseases are promoted by the …