ALS & FTD: TDP-43

Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) share a considerable proportion of characteristics including the same neuropathological substrate of abnormally phosphorylated TAR DNA binding protein (TDP-43). Here are the latest discoveries pertaining to ALS, FTD and TDP-43.

September 29, 2020
Review
Open Access

The Development of C9orf72-Related Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Disorders

Frontiers in Genetics
Qijie YangLu Shen
October 9, 2020
Open Access

Overexpression of UBQLN1 reduces neuropathology in the P497S UBQLN2 mouse model of ALS/FTD

Acta Neuropathologica Communications
Shaoteng WangMervyn J Monteiro
September 23, 2020

Isolated parkinsonism is an atypical presentation of GRN and C9orf72 gene mutations

Parkinsonism & Related Disorders
Fábio CarneiroIsabelle Le Ber
October 6, 2020
Open Access

Type I PRMT Inhibition Protects Against C9ORF72 Arginine-Rich Dipeptide Repeat Toxicity

Frontiers in Pharmacology
Alan S PremasiriFernando G Vieira
September 24, 2020

Inside minds, beneath diseases: social cognition in amyotrophic lateral sclerosis-frontotemporal spectrum disorder

Journal of Neurology, Neurosurgery, and Psychiatry
Patricia LilloLeonardo Cruz de Souza
September 28, 2020
Preprint
Open Access

Systemic Inflammation Causes Microglial Dysfunction With a Mixed Ad-like Pathology

ResearchSquare
Praveen Bathinilavinia Alberi
October 22, 2020

C9orf72-associated frontotemporal dementia in the Russian population

Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova
Yu A ShpilyukovaS N Illarioshkin
September 30, 2020
Open Access

TDP-43 dysfunction results in R-loop accumulation and DNA replication defects

Journal of Cell Science
Matthew WoodAlessandro Vindigni
October 7, 2020
Open Access

Monoclonal full-length antibody against TAR DNA-binding protein 43 reduces related proteinopathy in neurons

JCI Insight
Silvia PozziJean-Pierre Julien
October 8, 2020

Full-length TDP-43 and its C-terminal domain form filaments in vitro having non-amyloid properties

Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis
Claudia CapitiniFabrizio Chiti
October 11, 2020

Hemizygous deletion of Tbk1 worsens neuromuscular junction pathology in TDP-43G298S transgenic mice

Experimental Neurology
Kirsten SieverdingJochen H Weishaupt
October 1, 2020

Preclinical models of disease and multimorbidity with focus upon cardiovascular disease and dementia

Mechanisms of Ageing and Development
Osman ShabirSheila E Francis
October 1, 2020
Open Access

Stress Granule Assembly Can Facilitate but Is Not Required for TDP-43 Cytoplasmic Aggregation

Biomolecules
Nikita FernandesJ Ross Buchan
September 20, 2020

Necrosome-positive granulovacuolar degeneration is associated with TDP-43 pathological lesions in the hippocampus of ALS/FTLD cases

Neuropathology and Applied Neurobiology
E Van SchoorD R Thal
September 28, 2020
Open Access

Variable clinical phenotype in TBK1 mutations: case report of a novel mutation causing primary progressive aphasia and review of the literature

Neurobiology of Aging
Imogen J SwiftJonathan D Rohrer
October 21, 2020
Open Access

Withaferin-A Treatment Alleviates TAR DNA-Binding Protein-43 Pathology and Improves Cognitive Function in a Mouse Model of FTLD

Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics
Sunny KumarJean-Pierre Julien
September 16, 2020
Preprint
Open Access

Multiple pathways of toxicity induced by C9orf72 dipeptide repeat aggregates and G4C2 RNA in a cellular model

BioRxiv : the Preprint Server for Biology
F. FrottinMark S Hipp
October 22, 2020
Review
Open Access

The Peptidyl-prolyl Isomerase Pin1 in Neuronal Signaling: from Neurodevelopment to Neurodegeneration

Molecular Neurobiology
Francesca FagianiCristina Lanni

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