Thioridazine reverts the phenotype in cellular and Drosophila models of amyotrophic lateral sclerosis by enhancing TDP-43 aggregate clearance

Role of SNAREs in Neurodegenerative Diseases

(2021) Azzurra Margiotta   Cells

Detection of TAR DNA-binding protein 43 (TDP-43) oligomers as initial intermediate species during aggregate formation

(2019) Rachel L. French et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The mutational landscape of a prion-like domain

(2019) Benedetta Bolognesi et al.   Nature Communications

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

(2018) Yoshitaka Tamaki et al.   Scientific Reports

New routes in frontotemporal dementia drug discovery

(2017) Laura De Conti et al.   Expert Opinion on Drug Discovery

Monitoring and Measuring Autophagy

(2017) Saori R. Yoshii et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

The N-terminal dimerization is required for TDP-43 splicing activity

(2017) Lei-Lei Jiang et al.   Scientific Reports

UBQLN2 Mediates Autophagy-Independent Protein Aggregate Clearance by the Proteasome

(2016) Roland Hjerpe et al.   CELL

Mechanistic Insights into Hsp104 Potentiation

(2016) Mariana P. Torrente et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Quantification of the Relative Contributions of Loss-of-function and Gain-of-function Mechanisms in TAR DNA-binding Protein 43 (TDP-43) Proteinopathies

(2016) Roberta Cascella et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Physiological functions and pathobiology of TDP-43 and FUS/TLS proteins

(2016) Antonia Ratti et al.   JOURNAL OF NEUROCHEMISTRY

Decoding ALS: from genes to mechanism

(2016) J. Paul Taylor et al.   NATURE

In vitro prion-like behaviour of TDP-43 in ALS

(2016) Phillip Smethurst et al.   NEUROBIOLOGY OF DISEASE

Functional recovery in new mouse models of ALS/FTLD after clearance of pathological cytoplasmic TDP-43

(2015) Adam K. Walker et al.   ACTA NEUROPATHOLOGICA

An age-related reduction of brain TBPH/TDP-43 levels precedes the onset of locomotion defects in a Drosophila ALS model

(2015) L. Cragnaz et al.   NEUROSCIENCE

TDP-43 affects splicing profiles and isoform production of genes involved in the apoptotic and mitotic cellular pathways

(2015) Laura De Conti et al.   NUCLEIC ACIDS RESEARCH

TDP-43 repression of nonconserved cryptic exons is compromised in ALS-FTD

(2015) J. P. Ling et al.   SCIENCE

Guidelines for the use and interpretation of assays for monitoring autophagy in higher eukaryotes

(2014) Daniel J. Klionsky et al.   Autophagy

PPAR gamma activation is neuroprotective in a Drosophila model of ALS based on TDP-43

(2014) Archi Joardar et al.   HUMAN MOLECULAR GENETICS

TDP-43 loss of cellular function through aggregation requires additional structural determinants beyond its C-terminal Q/N prion-like domain

(2014) Mauricio Budini et al.   HUMAN MOLECULAR GENETICS

Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models

(2014) Sami J Barmada et al.   Nature Chemical Biology

Aggregate formation prevents dTDP-43 neurotoxicity in the Drosophila melanogaster eye

(2014) Lucia Cragnaz et al.   NEUROBIOLOGY OF DISEASE

Chronological requirements of TDP-43 function in synaptic organization and locomotive control

(2014) Giulia Romano et al.   NEUROBIOLOGY OF DISEASE

Axonal Transport of TDP-43 mRNA Granules Is Impaired by ALS-Causing Mutations

(2014) Nael H. Alami et al.   NEURON

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

(2014) C. Yang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Loss of TDP-43 causes age-dependent progressive motor neuron degeneration

(2013) Yohei Iguchi et al.   BRAIN

The dual functions of the extreme N-terminus of TDP-43 in regulating its biological activity and inclusion formation

(2013) Yong-Jie Zhang et al.   HUMAN MOLECULAR GENETICS

Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis

(2013) Shuo-Chien Ling et al.   NEURON

The Contribution of Lysosomotropism to Autophagy Perturbation

(2013) Roshan Ashoor et al.   PLoS One

Prion-like Properties of Pathological TDP-43 Aggregates from Diseased Brains

(2013) Takashi Nonaka et al.   Cell Reports

Cellular Model of TAR DNA-binding Protein 43 (TDP-43) Aggregation Based on Its C-terminal Gln/Asn-rich Region

(2012) Mauricio Budini et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Alteration of POLDIP3 Splicing Associated with Loss of Function of TDP-43 in Tissues Affected with ALS

(2012) Atsushi Shiga et al.   PLoS One

TDP-43: gumming up neurons through protein–protein and protein–RNA interactions

(2012) Emanuele Buratti et al.   TRENDS IN BIOCHEMICAL SCIENCES

A Seeding Reaction Recapitulates Intracellular Formation of Sarkosyl-insoluble Transactivation Response Element (TAR) DNA-binding Protein-43 Inclusions

(2011) Yoshiaki Furukawa et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

(2011) Han-Xiang Deng et al.   NATURE

Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration

(2011) Edward B. Lee et al.   NATURE REVIEWS NEUROSCIENCE

TDP-43 regulates global translational yield by splicing of exon junction complex component SKAR

(2011) Fabienne C. Fiesel et al.   NUCLEIC ACIDS RESEARCH

Mitochondrial dysfunction in ALS

(2011) Mauro Cozzolino et al.   PROGRESS IN NEUROBIOLOGY

Loss of murine TDP-43 disrupts motor function and plays an essential role in embryogenesis

(2010) Brian C. Kraemer et al.   ACTA NEUROPATHOLOGICA

Nuclear import impairment causes cytoplasmic trans-activation response DNA-binding protein accumulation and is associated with frontotemporal lobar degeneration

(2010) Agnes L. Nishimura et al.   BRAIN

The “Dying-Back” Phenomenon of Motor Neurons in ALS

(2010) Michal Dadon-Nachum et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia

(2010) Ian RA Mackenzie et al.   LANCET NEUROLOGY

Phosphorylation regulates proteasomal-mediated degradation and solubility of TAR DNA binding protein-43 C-terminal fragments

(2010) Yong-Jie Zhang et al.   Molecular Neurodegeneration

TDP-43-Based Animal Models of Neurodegeneration: New Insights into ALS Pathology and Pathophysiology

(2010) Iga Wegorzewska et al.   Neurodegenerative Diseases

A small-molecule scaffold induces autophagy in primary neurons and protects against toxicity in a Huntington disease model

(2010) A. S. Tsvetkov et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A high content screening assay for identifying lysosomotropic compounds

(2010) Sashi Nadanaciva et al.   TOXICOLOGY IN VITRO

Depletion of TDP-43 affectsDrosophila motoneuronsterminal synapsis and locomotive behavior

(2009) Fabian Feiguin et al.   FEBS LETTERS

TDP-43 Is a Developmentally Regulated Protein Essential for Early Embryonic Development

(2009) Chantelle F. Sephton et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Desmethylclomipramine induces the accumulation of autophagy markers by blocking autophagic flux

(2009) M. Rossi et al.   JOURNAL OF CELL SCIENCE

Amyotrophic lateral sclerosis, frontotemporal dementia and beyond: the TDP-43 diseases

(2009) Felix Geser et al.   JOURNAL OF NEUROLOGY

Functional mapping of the interaction between TDP-43 and hnRNP A2 in vivo

(2009) A. D'Ambrogio et al.   NUCLEIC ACIDS RESEARCH

Autophagy and the ubiquitin-proteasome system: Collaborators in neuroprotection

(2008) Natalia B. Nedelsky et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

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

(2008) Matthew J. Winton et al.   FEBS LETTERS

Structural determinants of the cellular localization and shuttling of TDP-43

(2008) Y. M. Ayala et al.   JOURNAL OF CELL SCIENCE

TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

(2008) J. Sreedharan et al.   SCIENCE