Molecular, functional, and pathological aspects of TDP-43 fragmentation

ALS-linked TDP-43M337V knock-in mice exhibit splicing deregulation without neurodegeneration

(2020) Seiji Watanabe et al.   Molecular Brain

A robust TDP-43 knock-in mouse model of ALS

(2020) Shih-Ling Huang et al.   Acta Neuropathologica Communications

The emerging role of α-synuclein truncation in aggregation and disease

(2020) Zachary A. Sorrentino et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Distribution of TDP-43 Pathology in Hippocampal Synaptic Relays Suggests Transsynaptic Propagation in Frontotemporal Lobar Degeneration

(2020) Pouya Jamshidi et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Distinct molecular patterns of TDP-43 pathology in Alzheimer’s disease: relationship with clinical phenotypes

(2020) Sandra O. Tomé et al.   Acta Neuropathologica Communications

Exosomal TAR DNA-binding protein-43 and neurofilaments in plasma of amyotrophic lateral sclerosis patients: A longitudinal follow-up study

(2020) Po-Chih Chen et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

ALS-implicated protein TDP-43 sustains levels of STMN2, a mediator of motor neuron growth and repair

(2019) Joseph R. Klim et al.   NATURE NEUROSCIENCE

Premature polyadenylation-mediated loss of stathmin-2 is a hallmark of TDP-43-dependent neurodegeneration

(2019) Ze’ev Melamed et al.   NATURE NEUROSCIENCE

Mutant TDP-43 Causes Early-Stage Dose-Dependent Motor Neuron Degeneration in a TARDBP Knockin Mouse Model of ALS

(2019) Sarah Y. Ebstein et al.   Cell Reports

Molecular Mechanisms of TDP-43 Misfolding and Pathology in Amyotrophic Lateral Sclerosis

(2019) Archana Prasad et al.   Frontiers in Molecular Neuroscience

Caspase-4 mediates cytoplasmic accumulation of TDP-43 in the primate brains

(2019) Peng Yin et al.   ACTA NEUROPATHOLOGICA

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

(2019) Rudolf C Hergesheimer et al.   BRAIN

The Pathobiology of TDP-43 C-Terminal Fragments in ALS and FTLD

(2019) Britt A. Berning et al.   Frontiers in Neuroscience

Mice deficient in the C-terminal domain of TAR DNA-binding protein 43 develop age-dependent motor dysfunction associated with impaired Notch1−Akt signaling pathway

(2019) Kohei Nishino et al.   Acta Neuropathologica Communications

Structural dissection of amyloid aggregates of TDP‐43 and its C‐terminal fragments TDP‐35 and TDP‐16

(2019) Jayakrishna Shenoy et al.   FEBS Journal

Shortened TDP43 isoforms upregulated by neuronal hyperactivity drive TDP43 pathology in ALS

(2019) Kaitlin Weskamp et al.   JOURNAL OF CLINICAL INVESTIGATION

Mouse models of ALS: Past, present and future

(2018) Cathleen Lutz   BRAIN RESEARCH

Raloxifene, a promising estrogen replacement, limits TDP-25 cell death by enhancing autophagy and suppressing apoptosis

(2018) Fuling Zhou et al.   BRAIN RESEARCH BULLETIN

Mice with endogenous TDP‐43 mutations exhibit gain of splicing function and characteristics of amyotrophic lateral sclerosis

(2018) Pietro Fratta et al.   EMBO JOURNAL

Tau Proteolysis in the Pathogenesis of Tauopathies: Neurotoxic Fragments and Novel Biomarkers

(2018) James P. Quinn et al.   JOURNAL OF ALZHEIMERS DISEASE

TDP-43 gains function due to perturbed autoregulation in a Tardbp knock-in mouse model of ALS-FTD

(2018) Matthew A. White et al.   NATURE NEUROSCIENCE

TDP-43 pathology disrupts nuclear pore complexes and nucleocytoplasmic transport in ALS/FTD

(2018) Ching-Chieh Chou et al.   NATURE NEUROSCIENCE

Active nuclear import and passive nuclear export are the primary determinants of TDP-43 localization

(2018) Emile S. Pinarbasi et al.   Scientific Reports

TDP43 nuclear export and neurodegeneration in models of amyotrophic lateral sclerosis and frontotemporal dementia

(2018) Hilary C. Archbold et al.   Scientific Reports

Alternative Splicing of Alpha- and Beta-Synuclein Genes Plays Differential Roles in Synucleinopathies

(2018) et al.   Genes

Tdp-25 Routing to Autophagy and Proteasome Ameliorates its Aggregation in Amyotrophic Lateral Sclerosis Target Cells

(2018) Maria Elena Cicardi et al.   Scientific Reports

Age-related deregulation of TDP-43 after stroke enhances NF-κB-mediated inflammation and neuronal damage

(2018) Sai Sampath Thammisetty et al.   Journal of Neuroinflammation

Chronic oxidative stress promotes GADD34-mediated phosphorylation of the TAR DNA-binding protein TDP-43, a modification linked to neurodegeneration

(2017) Catherine Wenhui Goh et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Heat Shock-induced Phosphorylation of TAR DNA-binding Protein 43 (TDP-43) by MAPK/ERK Kinase Regulates TDP-43 Function

(2017) Wen Li et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Point mutations in the N-terminal domain of transactive response DNA-binding protein 43 kDa (TDP-43) compromise its stability, dimerization, and functions

(2017) Miguel Mompeán et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

TDP-43 proteolysis is associated with astrocyte reactivity after traumatic brain injury in rodents

(2017) Chih-Yuan Huang et al.   JOURNAL OF NEUROIMMUNOLOGY

Functional and dynamic polymerization of the ALS-linked protein TDP-43 antagonizes its pathologic aggregation

(2017) Tariq Afroz et al.   Nature Communications

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

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

Depletion of TDP-43 decreases fibril and plaque β-amyloid and exacerbates neurodegeneration in an Alzheimer’s mouse model

(2016) Katherine D. LaClair et al.   ACTA NEUROPATHOLOGICA

Updated TDP-43 in Alzheimer’s disease staging scheme

(2016) Keith A. Josephs et al.   ACTA NEUROPATHOLOGICA

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

(2016) Hiroki Sasaguri et al.   BRAIN RESEARCH

The chaperone HSPB8 reduces the accumulation of truncated TDP-43 species in cells and protects against TDP-43-mediated toxicity

(2016) Valeria Crippa et al.   HUMAN MOLECULAR GENETICS

Overexpression of heat shock factor 1 maintains TAR DNA binding protein 43 solubility via induction of inducible heat shock protein 70 in cultured cells

(2016) Pei-Yi Lin et al.   JOURNAL OF NEUROSCIENCE RESEARCH

The Neurotoxic Tau45-230 Fragment Accumulates in Upper and Lower Motor Neurons in Amyotrophic Lateral Sclerosis Subjects

(2016) Claudia R Vintilescu et al.   MOLECULAR MEDICINE

Lentiviral vector-mediated overexpression of mutant ataxin-7 recapitulates SCA7 pathology and promotes accumulation of the FUS/TLS and MBNL1 RNA-binding proteins

(2016) Sandro Alves et al.   Molecular Neurodegeneration

Phosphorylated TDP-43 becomes resistant to cleavage by calpain: A regulatory role for phosphorylation in TDP-43 pathology of ALS/FTLD

(2016) Takenari Yamashita et al.   NEUROSCIENCE RESEARCH

Electrostatic Repulsion Governs TDP-43 C-terminal Domain Aggregation

(2016) Miguel Mompeán et al.   PLOS BIOLOGY

Structural analysis of disease-related TDP-43 D169G mutation: linking enhanced stability and caspase cleavage efficiency to protein accumulation

(2016) Chien-Hao Chiang et al.   Scientific Reports

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

(2016) Fuyuki Kametani et al.   Scientific Reports

Interaction of RNA with a C-terminal fragment of the amyotrophic lateral sclerosis-associated TDP43 reduces cytotoxicity

(2016) Akira Kitamura et al.   Scientific Reports

Low molecular weight species of TDP-43 generated by abnormal splicing form inclusions in amyotrophic lateral sclerosis and result in motor neuron death

(2015) Shangxi Xiao et al.   ACTA NEUROPATHOLOGICA

TDP-43 activates microglia through NF-κB and NLRP3 inflammasome

(2015) Weihua Zhao et al.   EXPERIMENTAL NEUROLOGY

TDP-35 sequesters TDP-43 into cytoplasmic inclusions through binding with RNA

(2015) Mei-Xia Che et al.   FEBS LETTERS

Reduced protein turnover mediates functional deficits in transgenic mice expressing the 25 kDa C-terminal fragment of TDP-43

(2015) Antonella Caccamo et al.   HUMAN MOLECULAR GENETICS

An insoluble frontotemporal lobar degeneration-associated TDP-43 C-terminal fragment causes neurodegeneration and hippocampus pathology in transgenic mice

(2015) Adam K. Walker et al.   HUMAN MOLECULAR GENETICS

Valproate Attenuates 25-kDa C-Terminal Fragment of TDP-43-Induced Neuronal Toxicity via Suppressing Endoplasmic Reticulum Stress and Activating Autophagy

(2015) Xuejing Wang et al.   International Journal of Biological Sciences

Traumatic brain injury causes frontotemporal dementia and TDP-43 proteolysis

(2015) H.-K. Wang et al.   NEUROSCIENCE

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

(2015) J. P. Ling et al.   SCIENCE

The cleavage pattern of TDP-43 determines its rate of clearance and cytotoxicity

(2015) Quan Li et al.   Nature Communications

Reduced cellular Ca2+ availability enhances TDP-43 cleavage by apoptotic caspases

(2014) Giovanni De Marco et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Dual Vulnerability of TDP-43 to Calpain and Caspase-3 Proteolysis after Neurotoxic Conditions and Traumatic Brain Injury

(2014) Zhihui Yang et al.   JOURNAL OF CEREBRAL BLOOD FLOW AND METABOLISM

Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models

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

Amyloid-β oligomers as a template for secondary amyloidosis in Alzheimer's disease

(2014) Marcos J. Guerrero-Muñoz et al.   NEUROBIOLOGY OF DISEASE

A new cellular model of pathological TDP-43: The neurotoxicity of stably expressed CTF25 of TDP-43 depends on the proteasome

(2014) Y. Liu et al.   NEUROSCIENCE

Neuronal degeneration, synaptic defects, and behavioral abnormalities in tau45-230 transgenic mice

(2014) A.E. Lang et al.   NEUROSCIENCE

TDP-43 Inclusion Bodies Formed in Bacteria Are Structurally Amorphous, Non-Amyloid and Inherently Toxic to Neuroblastoma Cells

(2014) Claudia Capitini et al.   PLoS One

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

CDC7 inhibition blocks pathological TDP-43 phosphorylation and neurodegeneration

(2013) Nicole F. Liachko et al.   ANNALS OF NEUROLOGY

Loss and gain of Drosophila TDP-43 impair synaptic efficacy and motor control leading to age-related neurodegeneration by loss-of-function phenotypes

(2013) Danielle C. Diaper et al.   HUMAN MOLECULAR GENETICS

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

The Truncated C-terminal RNA Recognition Motif of TDP-43 Protein Plays a Key Role in Forming Proteinaceous Aggregates

(2013) Yi-Ting Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Protein Quality Control System in Neurodegeneration: A Healing Company Hard to Beat but Failure is Fatal

(2013) Deepak Chhangani et al.   MOLECULAR NEUROBIOLOGY

Overexpression of ALS-Associated p.M337V Human TDP-43 in Mice Worsens Disease Features Compared to Wild-type Human TDP-43 Mice

(2013) Jonathan Janssens et al.   MOLECULAR NEUROBIOLOGY

Selective Forelimb Impairment in Rats Expressing a Pathological TDP-43 25 kDa C-terminal Fragment to Mimic Amyotrophic Lateral Sclerosis

(2013) Robert D Dayton et al.   MOLECULAR THERAPY

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

(2013) Shuo-Chien Ling et al.   NEURON

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

(2013) Takashi Nonaka et al.   Cell Reports

Coexistence of Huntington’s disease and amyotrophic lateral sclerosis: a clinicopathologic study

(2012) Mari Tada et al.   ACTA NEUROPATHOLOGICA

Wild type TDP-43 induces neuro-inflammation and alters APP metabolism in lentiviral gene transfer models

(2012) Alexander M. Herman et al.   EXPERIMENTAL NEUROLOGY

ALS-Associated Ataxin 2 PolyQ Expansions Enhance Stress-Induced Caspase 3 Activation and Increase TDP-43 Pathological Modifications

(2012) M. P. Hart et al.   JOURNAL OF NEUROSCIENCE

Does a loss of TDP-43 function cause neurodegeneration?

(2012) Zuo-Shang Xu   Molecular Neurodegeneration

The Aggregation and Neurotoxicity of TDP-43 and Its ALS-Associated 25 kDa Fragment Are Differentially Affected by Molecular Chaperones in Drosophila

(2012) Jenna M. Gregory et al.   PLoS One

Coaggregation of RNA-Binding Proteins in a Model of TDP-43 Proteinopathy with Selective RGG Motif Methylation and a Role for RRM1 Ubiquitination

(2012) Eric B. Dammer et al.   PLoS One

Autophagy activators rescue and alleviate pathogenesis of a mouse model with proteinopathies of the TAR DNA-binding protein 43

(2012) I.-F. Wang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Asparaginyl endopeptidase cleaves TDP-43 in brain

(2012) Jeremy H. Herskowitz et al.   PROTEOMICS

A role for calpain-dependent cleavage of TDP-43 in amyotrophic lateral sclerosis pathology

(2012) Takenari Yamashita et al.   Nature Communications

The self-interaction of native TDP-43 C terminus inhibits its degradation and contributes to early proteinopathies

(2012) I.-Fan Wang et al.   Nature Communications

Spinocerebellar ataxia type 2 (SCA2) is associated with TDP-43 pathology

(2011) Yasuko Toyoshima et al.   ACTA NEUROPATHOLOGICA

Molecular properties of TAR DNA binding protein-43 fragments are dependent upon its cleavage site

(2011) Yoshiaki Furukawa et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

β-Amyloid triggers ALS-associated TDP-43 pathology in AD models

(2011) Alexander M. Herman et al.   BRAIN RESEARCH

FTD and ALS: A Tale of Two Diseases

(2011) R. Ferrari et al.   Current Alzheimer Research

Understanding the role of TDP-43 and FUS/TLS in ALS and beyond

(2011) Sandrine Da Cruz et al.   CURRENT OPINION IN NEUROBIOLOGY

TDP-43 neurotoxicity and protein aggregation modulated by heat shock factor and insulin/IGF-1 signaling

(2011) Tao Zhang et al.   HUMAN MOLECULAR GENETICS

TAR DNA-binding protein 43 (TDP-43) regulates stress granule dynamics via differential regulation of G3BP and TIA-1

(2011) Karli K. McDonald et al.   HUMAN MOLECULAR GENETICS

A “Two-hit” Hypothesis for Inclusion Formation by Carboxyl-terminal Fragments of TDP-43 Protein Linked to RNA Depletion and Impaired Microtubule-dependent Transport

(2011) G. Scott Pesiridis et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

TDP-43-induced Death Is Associated with Altered Regulation of BIM and Bcl-xL and Attenuated by Caspase-mediated TDP-43 Cleavage

(2011) Hiroaki Suzuki et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

TDP-43 Autoregulation: Implications for Disease

(2011) Mauricio Budini et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

RNA targets of TDP-43 identified by UV-CLIP are deregulated in ALS

(2011) Shangxi Xiao et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Characterizing the RNA targets and position-dependent splicing regulation by TDP-43

(2011) James R Tollervey et al.   NATURE NEUROSCIENCE

Hyperphosphorylation as a Defense Mechanism to Reduce TDP-43 Aggregation

(2011) Huei-Ying Li et al.   PLoS One

Identification of Neuronal RNA Targets of TDP-43-containing Ribonucleoprotein Complexes

(2010) Chantelle F. Sephton et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Elevated expression of TDP-43 in the forebrain of mice is sufficient to cause neurological and pathological phenotypes mimicking FTLD-U

(2010) Kuen-Jer Tsai et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Biochemical and histopathological alterations in TAR DNA-binding protein-43 after acute ischemic stroke in rats

(2010) Masato Kanazawa et al.   JOURNAL OF NEUROCHEMISTRY

TDP-43 Mediates Degeneration in a Novel Drosophila Model of Disease Caused by Mutations in VCP/p97

(2010) G. P. Ritson et al.   JOURNAL OF NEUROSCIENCE

Phosphorylation Promotes Neurotoxicity in a Caenorhabditis elegans Model of TDP-43 Proteinopathy

(2010) N. F. Liachko et al.   JOURNAL OF NEUROSCIENCE

Wild-Type Human TDP-43 Expression Causes TDP-43 Phosphorylation, Mitochondrial Aggregation, Motor Deficits, and Early Mortality in Transgenic Mice

(2010) Y.-F. Xu et al.   JOURNAL OF NEUROSCIENCE

Progressive motor weakness in transgenic mice expressing human TDP-43

(2010) Nancy R. Stallings et al.   NEUROBIOLOGY OF DISEASE

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

(2010) Iga Wegorzewska et al.   Neurodegenerative Diseases

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

(2010) Aaron Voigt et al.   PLoS One

ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS

(2010) S.-C. Ling et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

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

(2010) Hans Wils et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Selective occurrence of TDP-43-immunoreactive inclusions in the lower motor neurons in Machado–Joseph disease

(2009) Chun-Feng Tan et al.   ACTA NEUROPATHOLOGICA

Protein quality control during aging involves recruitment of the macroautophagy pathway by BAG3

(2009) Martin Gamerdinger et al.   EMBO JOURNAL

Truncation and pathogenic mutations facilitate the formation of intracellular aggregates of TDP-43

(2009) Takashi Nonaka et al.   HUMAN MOLECULAR GENETICS

Degradation of TDP-43 and its pathogenic form by autophagy and the ubiquitin-proteasome system

(2009) Xiaoju Wang et al.   NEUROSCIENCE LETTERS

Structural insights into TDP-43 in nucleic-acid binding and domain interactions

(2009) P.-H. Kuo et al.   NUCLEIC ACIDS RESEARCH

TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration

(2009) I. Wegorzewska et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity

(2009) Y.-J. Zhang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Enrichment of C-Terminal Fragments in TAR DNA-Binding Protein-43 Cytoplasmic Inclusions in Brain but not in Spinal Cord of Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis

(2008) Lionel M. Igaz et al.   AMERICAN JOURNAL OF PATHOLOGY

Caspase-cleaved TAR DNA-binding protein-43 is a major pathological finding in Alzheimer's disease

(2008) Troy T. Rohn   BRAIN RESEARCH

ALS and FTLD: two faces of TDP-43 proteinopathy

(2008) R. M. Liscic et al.   EUROPEAN JOURNAL OF NEUROLOGY

Concomitant TAR-DNA-Binding Protein 43 Pathology Is Present in Alzheimer Disease and Corticobasal Degeneration but Not in Other Tauopathies

(2008) Kunihiro Uryu et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY