Quantification of the relative contributions of loss-of-function and gain-of-function mechanisms in TDP-43 proteinopathies (original) (raw)

Phosphorylated and ubiquitinated TDP-43 pathological inclusions in ALS and FTLD-U are recapitulated in SH-SY5Y cells

Emanuele Buratti

FEBS Letters, 2009

View PDFchevron_right

What is the key player in TDP-43 pathology in ALS: Disappearance from the nucleus or inclusion formation in the cytoplasm?

Osamu Onodera

Neurology and Clinical Neuroscience, 2013

View PDFchevron_right

TDP-43 Redistribution is an Early Event in Sporadic Amyotrophic Lateral Sclerosis

Alessia Pellerino

Brain Pathology, 2010

View PDFchevron_right

TDP-43 proteinopathies: a new wave of neurodegenerative diseases

Parvathi Menon

Journal of Neurology, Neurosurgery & Psychiatry

View PDFchevron_right

Molecular Neuropathology of TDP-43 Proteinopathies

Manuela Neumann

International Journal of Molecular Sciences, 2009

View PDFchevron_right

TDP-43 cytoplasmic inclusion formation is disrupted in C9orf72-associated amyotrophic lateral sclerosis/frontotemporal lobar degeneration

Seneshaw Asress

Brain Communications, 2019

View PDFchevron_right

On the development of markers for pathological TDP-43 in amyotrophic lateral sclerosis with and without dementia

Harald Hampel

Progress in Neurobiology, 2011

View PDFchevron_right

TDP-43 is a culprit in human neurodegeneration, and not just an innocent bystander

Elizabeth Fisher

Mammalian Genome, 2008

View PDFchevron_right

Wild type human TDP-43 potentiates ALS- linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS

耀翔 施

View PDFchevron_right

The Impact of ALS-Associated Genes hnRNPA1, MATR3, VCP and UBQLN2 on the Severity of TDP-43 Aggregation

Helena Motaln

Cells, 2020

View PDFchevron_right

Pathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disorders

Christine Van Broeckhoven

Human Molecular Genetics, 2013

View PDFchevron_right

The debated toxic role of aggregated TDP-43 in amyotrophic lateral sclerosis: a resolution in sight?

Anna Chami, Christian Andres

2019

View PDFchevron_right

Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis

Manuela Neumann

Science, 2006

View PDFchevron_right

The extreme N-terminus of TDP-43 mediates the cytoplasmic aggregation of TDP-43 and associated toxicity in vivo

Peter Bauer

Brain research, 2016

View PDFchevron_right

Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

Emanuele Buratti

Annals of Neurology, 2008

View PDFchevron_right

Expression of TDP-43 C-terminal Fragments in Vitro Recapitulates Pathological Features of TDP-43 Proteinopathies

Manuela Neumann

Journal of Biological Chemistry, 2009

View PDFchevron_right

Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice

Travis Unger

Journal of Clinical Investigation, 2011

View PDFchevron_right

TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration

Christine Van Broeckhoven

Proceedings of the National Academy of Sciences, 2010

View PDFchevron_right

Neuronal inclusion protein TDP-43 has no primary genetic role in FTD and ALS

Sebastiaan Engelborghs

Neurobiology of Aging, 2009

View PDFchevron_right

Partial loss of TDP-43 function causes phenotypes of amyotrophic lateral sclerosis

Leonardo J. Moya Aliaga

Proceedings of the National Academy of Sciences, 2014

View PDFchevron_right

Mass spectrometric analysis of accumulated TDP-43 in amyotrophic lateral sclerosis brains

Shigeo Murayama

Scientific reports, 2016

View PDFchevron_right

The role of TDP-43 in the pathogenesis of ALS and FTLD

Emanuele Buratti

2013

View PDFchevron_right

TDP-43/FUS in motor neuron disease: Complexity and challenges

Pavana m hegde

Progress in Neurobiology, 2016

View PDFchevron_right

Elimination of TDP-43 inclusions linked to amyotrophic lateral sclerosis by a misfolding-specific intrabody with dual proteolytic signals

Ryosuke Takahashi

Scientific reports, 2018

View PDFchevron_right

TDP-43 in skeletal muscle of patients affected with amyotrophic lateral sclerosis

Carla D'ascenzo, Corrado I Angelini, Emanuele Buratti

Amyotrophic Lateral Sclerosis, 2010

View PDFchevron_right

The molecular links between TDP-43 dysfunction and neurodegeneration

Emanuele Buratti

2009

View PDFchevron_right

TDP-43: multiple targets, multiple disease mechanisms?

michael sendtner

Nature Neuroscience, 2011

View PDFchevron_right

Multi-phaseted problems of TDP-43 in selective neuronal vulnerability in ALS

Kazuhide Asakawa

Cellular and Molecular Life Sciences

View PDFchevron_right

A90V TDP-43 variant results in the aberrant localization of TDP-43 in vitro

Anna M Karydas

FEBS Letters, 2008

View PDFchevron_right