ALS & FTD: TDP-43

TAR DNA-binding protein 43 (TDP-43) is a pathological protein identified in sporadic Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Dementia (FTD). Here are the latest discoveries pertaining to TDP-43 and these diseases.

October 13, 2021
Review
Open Access

Molecular Pathways Involved in Frontotemporal Lobar Degeneration with TDP-43 Proteinopathy: What Can We Learn from Proteomics?

International Journal of Molecular Sciences
Merel O MolElise G P Dopper
October 12, 2021
Preprint
Open Access

Loss of TBK1 activity leads to TDP-43 proteinopathy through lysosomal dysfunction in human motor neurons

BioRxiv : the Preprint Server for Biology
J. HaoKevin Eggan
October 12, 2021

A system-wide mislocalization of RNA-binding proteins in motor neurons is a new feature of ALS.

Neurobiology of Disease
Yu-Ju LiuYijuang Chern
October 11, 2021

Loss-of-function mutation in VCP mimics the characteristic pathology as in FTLD-TARDBP.

Autophagy
Abubakar Wani, Conrad C Weihl
October 7, 2021

Nuclear depletion of RNA-binding protein ELAVL3 (HuC) in sporadic and familial amyotrophic lateral sclerosis.

Acta Neuropathologica
Sandra Diaz-GarciaJohn Ravits
October 4, 2021
Open Access

Differential chemokine alteration in the variants of primary progressive aphasia-a role for neuroinflammation.

Journal of Neuroinflammation
Aitana Sogorb-EsteveJ. D. Rohrer
September 25, 2021

Clinico-genetic findings in 509 frontotemporal dementia patients.

Molecular Psychiatry
Matias WagnerJuliane Winkelmann
September 24, 2021
Review

Glial TDP-43 and TDP-43 induced glial pathology, focus on neurodegenerative proteinopathy syndromes.

Glia
Katherine E PraterSuman Jayadev
September 24, 2021
Preprint
Open Access

Concurrent tau pathologies in frontotemporal lobar degeneration with TDP-43 pathology

BioRxiv : the Preprint Server for Biology
S. KogaDennis W Dickson
September 22, 2021
Open Access

Age-related demethylation of the TDP-43 autoregulatory region in the human motor cortex.

Communications Biology
Yuka KoikeOsamu Onodera
September 22, 2021
Open Access

TDP-43 regulates GAD1 mRNA splicing and GABA signaling in Drosophila CNS.

Scientific Reports
Giulia RomanoFabian Feiguin
September 22, 2021

Role of genetics in amyotrophic lateral sclerosis: a large cohort study in Chinese mainland population.

Journal of Medical Genetics
Yong-Ping ChenHui-Fang Shang
September 22, 2021

Structural MRI Signatures in Genetic Presentations of the Frontotemporal Dementia-Motor Neuron Disease Spectrum.

Neurology
Edoardo Gioele SpinelliFederica Agosta
September 7, 2021
Review

Elucidating the Role of Cerebellar Synaptic Dysfunction in C9orf72-ALS/FTD - a Systematic Review and Meta-Analysis.

The Cerebellum
Aleksandra KaliszewskaNatalia Arias
September 2, 2021
Open Access

ALS-related FUS mutations alter axon growth in motoneurons and affect HuD/ELAVL4 and FMRP activity.

Communications Biology
Maria Giovanna GaroneAlessandro Rosa

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