[HTML][HTML] Modelling TDP-43 proteinopathy in Drosophila uncovers shared and neuron-specific targets across ALS and FTD relevant circuits

RK Godfrey, E Alsop, RT Bjork, BS Chauhan… - Acta neuropathologica …, 2023 - Springer
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) comprise a spectrum
of neurodegenerative diseases linked to TDP-43 proteinopathy, which at the cellular level, is …

Motor neurons and glia exhibit specific individualized responses to TDP-43 expression in a Drosophila model of amyotrophic lateral sclerosis

PS Estes, SG Daniel, AP Mccallum… - Disease models & …, 2013 - journals.biologists.com
Amyotrophic lateral sclerosis (ALS) is a fatal disease characterized by complex neuronal
and glial phenotypes. Recently, RNA-based mechanisms have been linked to ALS via RNA …

[HTML][HTML] Neuronal models of TDP-43 proteinopathy display reduced axonal translation, increased oxidative stress, and defective exocytosis

A Pisciottani, L Croci, F Lauria, C Marullo… - Frontiers in Cellular …, 2023 - ncbi.nlm.nih.gov
Amyotrophic lateral sclerosis (ALS) is a progressive, lethal neurodegenerative disease
mostly affecting people around 50–60 years of age. TDP-43, an RNA-binding protein …

Drosophila TDP-43 dysfunction in glia and muscle cells cause cytological and behavioural phenotypes that characterize ALS and FTLD

DC Diaper, Y Adachi, L Lazarou… - Human molecular …, 2013 - academic.oup.com
Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are
neurodegenerative disorders that are characterized by cytoplasmic aggregates and nuclear …

Wild-type and A315T mutant TDP-43 exert differential neurotoxicity in a Drosophila model of ALS

PS Estes, A Boehringer, R Zwick… - Human molecular …, 2011 - academic.oup.com
The RNA-binding protein TDP-43 has been linked to amyotrophic lateral sclerosis (ALS)
both as a causative locus and as a marker of pathology. With several missense mutations …

[PDF][PDF] Mutant TDP-43 causes early-stage dose-dependent motor neuron degeneration in a TARDBP knockin mouse model of ALS

SY Ebstein, I Yagudayeva, NA Shneider - Cell reports, 2019 - cell.com
Rare mutations in TARDBP, the gene encoding TDP-43, cause amyotrophic lateral sclerosis
(ALS), and TDP-43 pathology is seen in a large majority of ALS patients, suggesting a …

[HTML][HTML] TDP-43 prevents retrotransposon activation in the Drosophila motor system through regulation of Dicer-2 activity

G Romano, R Klima, F Feiguin - BMC biology, 2020 - Springer
Background Mutations in the small RNA-binding protein TDP-43 lead to the formation of
insoluble cytoplasmic aggregates that have been associated with the onset and progression …

A novel Drosophila model of TDP-43 proteinopathies: N-terminal sequences combined with the Q/N domain induce protein functional loss and locomotion defects

S Langellotti, V Romano, G Romano… - Disease Models & …, 2016 - journals.biologists.com
Transactive response DNA-binding protein 43 kDa (TDP-43, also known as TBPH in
Drosophila melanogaster and TARDBP in mammals) is the main protein component of the …

[HTML][HTML] TDP-43-Mediated Neuron Loss In Vivo Requires RNA-Binding Activity

A Voigt, D Herholz, FC Fiesel, K Kaur, D Müller… - PloS one, 2010 - journals.plos.org
Alteration and/or mutations of the ribonucleoprotein TDP-43 have been firmly linked to
human neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and …

[PDF][PDF] TDP-43 loss-of-function causes neuronal loss due to defective steroid receptor-mediated gene program switching in Drosophila

LV Broeck, M Naval-Sanchez, Y Adachi, D Diaper… - Cell reports, 2013 - cell.com
TDP-43 proteinopathy is strongly implicated in the pathogenesis of amyotrophic lateral
sclerosis and related neurodegenerative disorders. Whether TDP-43 neurotoxicity is caused …