TDP-43 induces mitochondrial damage and activates the mitochondrial unfolded protein response

Quantitative assessment of the degradation of aggregated TDP-43 mediated by the ubiquitin proteasome system and macroautophagy

(2017) Roberta Cascella et al.   FASEB JOURNAL

Sex specific activation of the ERα axis of the mitochondrial UPR (UPRmt) in the G93A-SOD1 mouse model of familial ALS

(2017) Amanjot K Riar et al.   HUMAN MOLECULAR GENETICS

The mitochondrial unfolded protein response: Signaling from the powerhouse

(2017) Mohammed A. Qureshi et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mutant TDP-43 does not impair mitochondrial bioenergetics in vitro and in vivo

(2017) Hibiki Kawamata et al.   Molecular Neurodegeneration

Cytosolic proteostasis through importing of misfolded proteins into mitochondria

(2017) Linhao Ruan et al.   NATURE

Truncation of the TAR DNA-binding protein 43 is not a prerequisite for cytoplasmic relocalization, and is suppressed by caspase inhibition and by introduction of the A90V sequence variant

(2017) Heike J. Wobst et al.   PLoS One

Activation of the mitochondrial unfolded protein response promotes longevity and dopamine neuron survival in Parkinson’s disease models

(2017) Jason F. Cooper et al.   Scientific Reports

Evidence for Mitochondrial UPR Gene Activation in Familial and Sporadic Alzheimer's Disease

(2016) John S. Beck et al.   Current Alzheimer Research

The Transcription Factor ATF5 Mediates a Mammalian Mitochondrial UPR

(2016) Christopher J. Fiorese et al.   CURRENT BIOLOGY

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

(2016) Antonia Ratti et al.   JOURNAL OF NEUROCHEMISTRY

Mitoguardin Regulates Mitochondrial Fusion through MitoPLD and Is Required for Neuronal Homeostasis

(2016) Yongping Zhang et al.   MOLECULAR CELL

The inhibition of TDP-43 mitochondrial localization blocks its neuronal toxicity

(2016) Wenzhang Wang et al.   NATURE MEDICINE

Mitochondria and endoplasmic reticulum crosstalk in amyotrophic lateral sclerosis

(2016) Giovanni Manfredi et al.   NEUROBIOLOGY OF DISEASE

Mitochondrial Cristae: Where Beauty Meets Functionality

(2016) Sara Cogliati et al.   TRENDS IN BIOCHEMICAL SCIENCES

Gene-specific mitochondria dysfunctions in human TARDBP and C9ORF72 fibroblasts

(2016) Elisa Onesto et al.   Acta Neuropathologica Communications

CODAS Syndrome Is Associated with Mutations of LONP1, Encoding Mitochondrial AAA+ Lon Protease

(2015) Kevin A. Strauss et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Oxr1 improves pathogenic cellular features of ALS-associated FUS and TDP-43 mutations

(2015) M. J. Finelli 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

New roles for mitochondrial proteases in health, ageing and disease

(2015) Pedro M. Quirós et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Studies of alternative isoforms provide insight into TDP-43 autoregulation and pathogenesis

(2015) S. D'Alton et al.   RNA

ALS Patient Stem Cells for Unveiling Disease Signatures of Motoneuron Susceptibility: Perspectives on the Deadly Mitochondria, ER Stress and Calcium Triad

(2015) Anjoscha Kaus et al.   Frontiers in Cellular Neuroscience

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

(2015) Quan Li et al.   Nature Communications

Oxidative stress and Parkinson’s disease

(2015) Javier Blesa et al.   Frontiers in Neuroanatomy

FUS Interacts with HSP60 to Promote Mitochondrial Damage

(2015) Jianwen Deng et al.   PLoS Genetics

Reactive oxygen species and mitochondria: A nexus of cellular homeostasis

(2015) Joe Dan Dunn et al.   Redox Biology

Frontotemporal lobar degeneration: defining phenotypic diversity through personalized medicine

(2014) David J. Irwin et al.   ACTA NEUROPATHOLOGICA

An ALS-mutant TDP-43 neurotoxic peptide adopts an anti-parallel β-structure and induces TDP-43 redistribution

(2014) Li Zhu et al.   HUMAN MOLECULAR GENETICS

Mitochondrial Dysfunction and Decrease in Body Weight of a Transgenic Knock-in Mouse Model for TDP-43

(2014) Carola Stribl et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

UBE2E Ubiquitin-conjugating Enzymes and Ubiquitin Isopeptidase Y Regulate TDP-43 Protein Ubiquitination

(2014) Friederike Hans et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Differential roles of the ubiquitin proteasome system and autophagy in the clearance of soluble and aggregated TDP-43 species

(2014) E. L. Scotter et al.   JOURNAL OF CELL SCIENCE

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

Disease-Associated Mutations of TDP-43 Promote Turnover of the Protein Through the Proteasomal Pathway

(2014) Wataru Araki et al.   MOLECULAR NEUROBIOLOGY

Frontotemporal lobar degeneration—building on breakthroughs

(2014) Julie van der Zee et al.   Nature Reviews Neurology

Oxidative stress and mitochondrial dysfunction in Alzheimer's disease

(2013) Xinglong Wang et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Mitochondrial Cristae Shape Determines Respiratory Chain Supercomplexes Assembly and Respiratory Efficiency

(2013) Sara Cogliati et al.   CELL

The ALS disease-associated mutant TDP-43 impairs mitochondrial dynamics and function in motor neurons

(2013) Wenzhang Wang et al.   HUMAN MOLECULAR GENETICS

Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse models

(2013) Jordi Magrané et al.   HUMAN MOLECULAR GENETICS

Controversies and priorities in amyotrophic lateral sclerosis

(2013) Martin R Turner et al.   LANCET NEUROLOGY

Fused in sarcoma (FUS): An oncogene goes awry in neurodegeneration

(2013) Dorothee Dormann et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Mitonuclear protein imbalance as a conserved longevity mechanism

(2013) Riekelt H. Houtkooper et al.   NATURE

State of play in amyotrophic lateral sclerosis genetics

(2013) Alan E Renton et al.   NATURE NEUROSCIENCE

The changing scene of amyotrophic lateral sclerosis

(2013) Wim Robberecht et al.   NATURE REVIEWS NEUROSCIENCE

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

(2013) Shuo-Chien Ling et al.   NEURON

The mitochondrial ATP-dependent Lon protease: a novel target in lymphoma death mediated by the synthetic triterpenoid CDDO and its derivatives

(2012) S. H. Bernstein et al.   BLOOD

Mitochondrial dysfunction in human TDP-43 transfected NSC34 cell lines and the protective effect of dimethoxy curcumin

(2012) Jinsheng Lu et al.   BRAIN RESEARCH BULLETIN

Autoregulation of TDP-43 mRNA levels involves interplay between transcription, splicing, and alternative polyA site selection

(2012) S. E. Avendano-Vazquez et al.   GENES & DEVELOPMENT

Mitochondria and ALS: Implications from novel genes and pathways

(2012) Mauro Cozzolino et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Assessment of mitochondrial respiratory chain enzymatic activities on tissues and cultured cells

(2012) Marco Spinazzi et al.   Nature Protocols

Asparaginyl endopeptidase cleaves TDP-43 in brain

(2012) Jeremy H. Herskowitz et al.   PROTEOMICS

Mitochondrial Import Efficiency of ATFS-1 Regulates Mitochondrial UPR Activation

(2012) A. M. Nargund et al.   SCIENCE

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

(2012) Takenari Yamashita et al.   Nature Communications

In Vivo Mapping of Hydrogen Peroxide and Oxidized Glutathione Reveals Chemical and Regional Specificity of Redox Homeostasis

(2011) Simone C. Albrecht et al.   Cell Metabolism

Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration

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

An ALS-associated mutation affecting TDP-43 enhances protein aggregation, fibril formation and neurotoxicity

(2011) Weirui Guo et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Three Distinct Roles for Notch in Drosophila R7 Photoreceptor Specification

(2011) Andrew Tomlinson et al.   PLOS BIOLOGY

Expression of human FUS protein in Drosophila leads to progressive neurodegeneration

(2011) Yanbo Chen et al.   Protein & Cell

Ubiquilin Modifies TDP-43 Toxicity in a Drosophila Model of Amyotrophic Lateral Sclerosis (ALS)

(2010) Keith A. Hanson et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Cytoplasmic Mislocalization of TDP-43 Is Toxic to Neurons and Enhanced by a Mutation Associated with Familial Amyotrophic Lateral Sclerosis

(2010) S. J. Barmada 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

TAR DNA-binding protein 43 in neurodegenerative disease

(2010) Alice S. Chen-Plotkin et al.   Nature Reviews Neurology

A Drosophila model for TDP-43 proteinopathy

(2010) Y. Li et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice

(2010) X. Shan et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

(2009) Xiaoju Wang et al.   NEUROSCIENCE LETTERS

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

How mitochondria produce reactive oxygen species

(2008) Michael P. Murphy   BIOCHEMICAL JOURNAL

Cristae formation—linking ultrastructure and function of mitochondria

(2008) Michael Zick et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Potentiation of Amyotrophic Lateral Sclerosis (ALS)-associated TDP-43 Aggregation by the Proteasome-targeting Factor, Ubiquilin 1

(2008) Sang Hwa Kim et al.   JOURNAL OF BIOLOGICAL CHEMISTRY