The ER mitochondria calcium cycle and ER stress response as therapeutic targets in amyotrophic lateral sclerosis

Neuronal-Specific Overexpression of a Mutant Valosin-Containing Protein Associated with IBMPFD Promotes Aberrant Ubiquitin and TDP-43 Accumulation and Cognitive Dysfunction in Transgenic Mice

(2013) Carlos J. Rodriguez-Ortiz et al.   AMERICAN JOURNAL OF PATHOLOGY

Calcium-dependent protein folding in amyotrophic lateral sclerosis

(2013) Tino Prell et al.   CELL CALCIUM

Overexpression of human mutated G93A SOD1 changes dynamics of the ER mitochondria calcium cycle specifically in mouse embryonic motor neurons

(2013) Janin Lautenschläger et al.   EXPERIMENTAL NEUROLOGY

S[+] Apomorphine is a CNS penetrating activator of the Nrf2-ARE pathway with activity in mouse and patient fibroblast models of amyotrophic lateral sclerosis

(2013) Richard J. Mead et al.   FREE RADICAL BIOLOGY AND MEDICINE

Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1

(2013) S. Pickles et al.   HUMAN MOLECULAR GENETICS

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

Functional and morphological impact of ER stress on mitochondria

(2013) Kayleen Vannuvel et al.   JOURNAL OF CELLULAR PHYSIOLOGY

Optineurin suppression causes neuronal cell death via NF-κB pathway

(2013) Mayumi Akizuki et al.   JOURNAL OF NEUROCHEMISTRY

Calcium Channel Agonists Protect against Neuromuscular Dysfunction in a Genetic Model of TDP-43 Mutation in ALS

(2013) G. A. B. Armstrong et al.   JOURNAL OF NEUROSCIENCE

Small Peptides against the Mutant SOD1/Bcl-2 Toxic Mitochondrial Complex Restore Mitochondrial Function and Cell Viability in Mutant SOD1-Mediated ALS

(2013) W. Tan et al.   JOURNAL OF NEUROSCIENCE

Enhancing Mitochondrial Calcium Buffering Capacity Reduces Aggregation of Misfolded SOD1 and Motor Neuron Cell Death without Extending Survival in Mouse Models of Inherited Amyotrophic Lateral Sclerosis

(2013) P. A. Parone et al.   JOURNAL OF NEUROSCIENCE

Selective mitochondrial Ca2+uptake deficit in disease endstage vulnerable motoneurons of the SOD1G93Amouse model of amyotrophic lateral sclerosis

(2013) Andrea Fuchs et al.   JOURNAL OF PHYSIOLOGY-LONDON

The modest impact of transcription factor Nrf2 on the course of disease in an ALS animal model

(2013) Yansu Guo et al.   LABORATORY INVESTIGATION

Dexpramipexole versus placebo for patients with amyotrophic lateral sclerosis (EMPOWER): a randomised, double-blind, phase 3 trial

(2013) Merit E Cudkowicz et al.   LANCET NEUROLOGY

Melatonin inhibits the caspase-1/cytochrome c/caspase-3 cell death pathway, inhibits MT1 receptor loss and delays disease progression in a mouse model of amyotrophic lateral sclerosis

(2013) Yi Zhang et al.   NEUROBIOLOGY OF DISEASE

Genetic ablation of phospholipase C delta 1 increases survival in SOD1G93A mice

(2013) Kim A. Staats et al.   NEUROBIOLOGY OF DISEASE

Pharmacological reduction of ER stress protects against TDP-43 neuronal toxicity in vivo

(2013) Alexandra Vaccaro et al.   NEUROBIOLOGY OF DISEASE

Neuroprotective effects of the Sigma-1 receptor (S1R) agonist PRE-084, in a mouse model of motor neuron disease not linked to SOD1 mutation

(2013) Marco Peviani et al.   NEUROBIOLOGY OF DISEASE

Pathogenic VCP Mutations Induce Mitochondrial Uncoupling and Reduced ATP Levels

(2013) Fernando Bartolome et al.   NEURON

Clinicopathologic features of autosomal recessive amyotrophic lateral sclerosis associated with optineurin mutation

(2013) Masaki Kamada et al.   NEUROPATHOLOGY

Intraperitoneal melatonin is not neuroprotective in the G93ASOD1 transgenic mouse model of familial ALS and may exacerbate neurodegeneration

(2013) Efthimios Dardiotis et al.   NEUROSCIENCE LETTERS

SA4503, a sigma-1 receptor agonist, suppresses motor neuron damage in in vitro and in vivo amyotrophic lateral sclerosis models

(2013) Yoko Ono et al.   NEUROSCIENCE LETTERS

SOD1 and DJ-1 Converge at Nrf2 Pathway: A Clue for Antioxidant Therapeutic Potential in Neurodegeneration

(2013) Pamela Milani et al.   Oxidative Medicine and Cellular Longevity

The role of heat shock proteins in Amyotrophic Lateral Sclerosis: The therapeutic potential of Arimoclomol

(2013) Bernadett Kalmar et al.   PHARMACOLOGY & THERAPEUTICS

Molecular Chaperone Mediated Late-Stage Neuroprotection in the SOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis

(2013) Sergey S. Novoselov et al.   PLoS One

ALS-Associated TDP-43 Induces Endoplasmic Reticulum Stress, Which Drives Cytoplasmic TDP-43 Accumulation and Stress Granule Formation

(2013) Adam K. Walker et al.   PLoS One

The C9orf72 GGGGCC Repeat Is Translated into Aggregating Dipeptide-Repeat Proteins in FTLD/ALS

(2013) K. Mori et al.   SCIENCE

Calcium, mitochondria, and the pathogenesis of ALS: the good, the bad, and the ugly

(2013) Manoj Kumar Jaiswal   Frontiers in Cellular Neuroscience

Benzothiazepine CGP37157 and Its Isosteric 2′-Methyl Analogue Provide Neuroprotection and Block Cell Calcium Entry

(2012) Laura González-Lafuente et al.   ACS Chemical Neuroscience

Endoplasmic reticulum stress and the ER mitochondria calcium cycle in amyotrophic lateral sclerosis

(2012) Janin Lautenschlaeger et al.   Amyotrophic Lateral Sclerosis

Treatment with a coinducer of the heat shock response delays muscle denervation in the SOD1-G93A mouse model of amyotrophic lateral sclerosis

(2012) Bernadett Kalmar et al.   Amyotrophic Lateral Sclerosis

Neuronal overexpression of IP3 receptor 2 is detrimental in mutant SOD1 mice

(2012) Kim A. Staats et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

TDP-43 aggregation in neurodegeneration: Are stress granules the key?

(2012) Colleen M. Dewey et al.   BRAIN RESEARCH

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

Toward an integrative view of Optineurin functions

(2012) David Kachaner et al.   CELL CYCLE

Physiological Roles of the Permeability Transition Pore

(2012) Catherine Brenner et al.   CIRCULATION RESEARCH

The unfolded protein response in models of human mutant G93A amyotrophic lateral sclerosis

(2012) T. Prell et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

Amyotrophic lateral sclerosis-associated mutant VAPBP56S perturbs calcium homeostasis to disrupt axonal transport of mitochondria

(2012) G. M. Morotz et al.   HUMAN MOLECULAR GENETICS

RNA-binding ability of FUS regulates neurodegeneration, cytoplasmic mislocalization and incorporation into stress granules associated with FUS carrying ALS-linked mutations

(2012) J. G. Daigle et al.   HUMAN MOLECULAR GENETICS

High-content RNAi screening identifies the Type 1 inositol triphosphate receptor as a modifier of TDP-43 localization and neurotoxicity

(2012) Sang Hwa Kim et al.   HUMAN MOLECULAR GENETICS

Ataxin-2 interacts with FUS and intermediate-length polyglutamine expansions enhance FUS-related pathology in amyotrophic lateral sclerosis

(2012) Manal A. Farg et al.   HUMAN MOLECULAR GENETICS

Accelerated Disease Onset with Stabilized Familial Amyotrophic Lateral Sclerosis (ALS)-linked Mutant TDP-43 Proteins

(2012) Shoji Watanabe et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Endoplasmic reticulum–mitochondria contacts: function of the junction

(2012) Ashley A. Rowland et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Olesoxime delays muscle denervation, astrogliosis, microglial activation and motoneuron death in an ALS mouse model

(2012) C. Sunyach et al.   NEUROPHARMACOLOGY

Dantrolene is neuroprotective in vitro, but does not affect survival in SOD1G93A mice

(2012) K.A. Staats et al.   NEUROSCIENCE

Full-length TDP-43 and its C-terminal fragments activate mitophagy in NSC34 cell line

(2012) Kun Hong et al.   NEUROSCIENCE LETTERS

Sigma-1R Agonist Improves Motor Function and Motoneuron Survival in ALS Mice

(2012) Renzo Mancuso et al.   Neurotherapeutics

C9orf72 hexanucleotide repeat associated with amyotrophic lateral sclerosis and frontotemporal dementia forms RNA G-quadruplexes

(2012) Pietro Fratta et al.   Scientific Reports

Protein disulfide isomerase-immunopositive inclusions in patients with amyotrophic lateral sclerosis

(2011) Yasuyuki Honjo et al.   Amyotrophic Lateral Sclerosis

Multitasking in the mitochondrion by the ATP-dependent Lon protease

(2011) Sundararajan Venkatesh et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

G37R SOD1 mutant alters mitochondrial complex I activity, Ca2+ uptake and ATP production

(2011) Evelyne Coussee et al.   CELL CALCIUM

VAPB interacts with the mitochondrial protein PTPIP51 to regulate calcium homeostasis

(2011) Kurt J. De Vos et al.   HUMAN MOLECULAR GENETICS

Neurotoxic 43-kDa TAR DNA-binding Protein (TDP-43) Triggers Mitochondrion-dependent Programmed Cell Death in Yeast

(2011) Ralf J. Braun et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Diacetylbis(N(4)-methylthiosemicarbazonato) Copper(II) (CuII(atsm)) Protects against Peroxynitrite-induced Nitrosative Damage and Prolongs Survival in Amyotrophic Lateral Sclerosis Mouse Model

(2011) Cynthia P. W. Soon 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

Involvement of Bid and caspase-2 in endoplasmic reticulum stress- and oxidative stress-induced retinal ganglion cell death

(2011) Rumi Uchibayashi et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Heat shock transcription factor 1 as a therapeutic target in neurodegenerative diseases

(2011) Daniel W. Neef et al.   NATURE REVIEWS DRUG DISCOVERY

Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis

(2011) Laura Ferraiuolo et al.   Nature Reviews Neurology

Bcl2-A1 interacts with pro-caspase-3: Implications for amyotrophic lateral sclerosis

(2011) Ciro Iaccarino et al.   NEUROBIOLOGY OF DISEASE

Calcium dysregulation, mitochondrial pathology and protein aggregation in a culture model of amyotrophic lateral sclerosis: Mechanistic relationship and differential sensitivity to intervention

(2011) Miranda L. Tradewell et al.   NEUROBIOLOGY OF DISEASE

Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS

(2011) Mariely DeJesus-Hernandez et al.   NEURON

A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD

(2011) Alan E. Renton et al.   NEURON

Metallothionein-III prevents neuronal death and prolongs life span in amyotrophic lateral sclerosis model mice

(2011) K. Hashimoto et al.   NEUROSCIENCE

Mitochondrial dysfunction in ALS

(2011) Mauro Cozzolino et al.   PROGRESS IN NEUROBIOLOGY

Uridine ameliorates the pathological phenotype in transgenic G93A-ALS mice

(2010) Daniel J. Amante et al.   Amyotrophic Lateral Sclerosis

The epidemiology and treatment of ALS: Focus on the heterogeneity of the disease and critical appraisal of therapeutic trials

(2010) Ettore Beghi et al.   Amyotrophic Lateral Sclerosis

SOD1 targeted to the mitochondrial intermembrane space prevents motor neuropathy in the Sod1 knockout mouse

(2010) Lindsey R. Fischer et al.   BRAIN

Calcium dysregulation in amyotrophic lateral sclerosis

(2010) Julian Grosskreutz et al.   CELL CALCIUM

The sites and topology of mitochondrial superoxide production

(2010) Martin D. Brand   EXPERIMENTAL GERONTOLOGY

The molecular identity of the mitochondrial Ca2+ sequestration system

(2010) Anatoly A. Starkov   FEBS Journal

Mitochondrial Ca2+ sequestration and precipitation revisited

(2010) Christos Chinopoulos et al.   FEBS Journal

Calcium-dependent mitochondrial function and dysfunction in neurons

(2010) Natalia B. Pivovarova et al.   FEBS Journal

Advanced age-related denervation and fiber-type grouping in skeletal muscle of SOD1 knockout mice

(2010) Tatiana Y. Kostrominova   FREE RADICAL BIOLOGY AND MEDICINE

ALS-linked mutant SOD1 damages mitochondria by promoting conformational changes in Bcl-2

(2010) Steve Pedrini et al.   HUMAN MOLECULAR GENETICS

AAV-mediated expression of wild-type and ALS-linked mutant VAPB selectively triggers death of motoneurons through a Ca2+-dependent ER-associated pathway

(2010) Karine Langou et al.   JOURNAL OF NEUROCHEMISTRY

Protein Disulfide Isomerase and the Endoplasmic Reticulum in Amyotrophic Lateral Sclerosis

(2010) A. K. Walker   JOURNAL OF NEUROSCIENCE

Functional Relationship between Protein Disulfide Isomerase Family Members during the Oxidative Folding of Human Secretory Proteins

(2010) Lori A. Rutkevich et al.   MOLECULAR BIOLOGY OF THE CELL

ALS spinal neurons show varied and reduced mtDNA gene copy numbers and increased mtDNA gene deletions

(2010) Paula M Keeney et al.   Molecular Neurodegeneration

Misfolded Mutant SOD1 Directly Inhibits VDAC1 Conductance in a Mouse Model of Inherited ALS

(2010) Adrian Israelson et al.   NEURON

Mutant TAR DNA-binding protein-43 induces oxidative injury in motor neuron-like cell

(2010) W. Duan et al.   NEUROSCIENCE

Deregulation of the hypoxia inducible factor-1α pathway in monocytes from sporadic amyotrophic lateral sclerosis patients

(2010) C. Moreau et al.   NEUROSCIENCE

Tar DNA Binding Protein-43 (TDP-43) Associates with Stress Granules: Analysis of Cultured Cells and Pathological Brain Tissue

(2010) Liqun Liu-Yesucevitz et al.   PLoS One

IP3 Receptors: Toward Understanding Their Activation

(2010) C. W. Taylor et al.   Cold Spring Harbor Perspectives in Biology

Ryanodine Receptors: Structure, Expression, Molecular Details, and Function in Calcium Release

(2010) J. T. Lanner et al.   Cold Spring Harbor Perspectives in Biology

Phase II trial of CoQ10 for ALS finds insufficient evidence to justify phase III

(2009) Petra Kaufmann et al.   ANNALS OF NEUROLOGY

Redox Modifier Genes and Pathways in Amyotrophic Lateral Sclerosis

(2009) Barrie J. Carter et al.   ANTIOXIDANTS & REDOX SIGNALING

Characteristics and possible functions of mitochondrial Ca2+ transport mechanisms

(2009) Thomas E. Gunter et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Cyclophilin D in mitochondrial pathophysiology

(2009) Valentina Giorgio et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

The mitochondrial permeability transition pore: A molecular target for amyotrophic lateral sclerosis therapy

(2009) Lee J. Martin   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Impairment of mitochondrial calcium handling in a mtSOD1 cell culture model of motoneuron disease

(2009) Manoj Jaiswal et al.   BMC NEUROSCIENCE

Functions and pathologies of BiP and its interaction partners

(2009) J. Dudek et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

The mitochondrial permeability transition pore in motor neurons: Involvement in the pathobiology of ALS mice

(2009) Lee J. Martin et al.   EXPERIMENTAL NEUROLOGY

Crosstalk signaling between mitochondrial Ca2+ and ROS

(2009) Robert et al.   Frontiers in Bioscience-Landmark

Voltage-dependent Anion Channel 1-based Peptides Interact with Bcl-2 to Prevent Antiapoptotic Activity

(2009) Nir Arbel et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A CaPful of mechanisms regulating the mitochondrial permeability transition

(2009) Fabio Di Lisa et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

A role for motoneuron subtype–selective ER stress in disease manifestations of FALS mice

(2009) Smita Saxena et al.   NATURE NEUROSCIENCE

Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis

(2009) T. J. Kwiatkowski et al.   SCIENCE

The expression of metallothioneins is diminished in the spinal cords of patients with sporadic ALS

(2008) Isao Hozumi et al.   Amyotrophic Lateral Sclerosis

The Identity and Regulation of the Mitochondrial Permeability Transition Pore

(2008) Magdalena Juhaszova et al.   Annals of the New York Academy of Sciences

How mitochondria produce reactive oxygen species

(2008) Michael P. Murphy   BIOCHEMICAL JOURNAL

Recent progress in elucidating the molecular mechanism of the mitochondrial permeability transition pore

(2008) Anna W.C. Leung et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Inhibition of endoplasmic reticulum stress counteracts neuronal cell death and protein aggregation caused by N-terminal mutant huntingtin proteins

(2008) Sami Reijonen et al.   EXPERIMENTAL CELL RESEARCH

The mechanisms of calcium homeostasis and signalling in the lens

(2008) Jeremy D. Rhodes et al.   EXPERIMENTAL EYE RESEARCH

Cysteine-674 oxidation and degradation of sarcoplasmic reticulum Ca2+ ATPase in diabetic pig aorta

(2008) Jia Ying et al.   FREE RADICAL BIOLOGY AND MEDICINE

Isolated CytochromecOxidase Deficiency in G93A SOD1 Mice Overexpressing CCS Protein

(2008) Marjatta Son et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Disturbance of Nuclear and Cytoplasmic TAR DNA-binding Protein (TDP-43) Induces Disease-like Redistribution, Sequestration, and Aggregate Formation

(2008) Matthew J. Winton et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Late stage treatment with arimoclomol delays disease progression and prevents protein aggregation in the SOD1G93A mouse model of ALS

(2008) Bernadett Kalmar et al.   JOURNAL OF NEUROCHEMISTRY

Caspase-2 Cleavage of BID Is a Critical Apoptotic Signal Downstream of Endoplasmic Reticulum Stress

(2008) J.-P. Upton et al.   MOLECULAR AND CELLULAR BIOLOGY

Cu/Zn Superoxide Dismutase Typical for Familial Amyotrophic Lateral Sclerosis Increases the Vulnerability of Mitochondria and Perturbs Ca2+ Homeostasis in SOD1G93A Mice

(2008) M. K. Jaiswal et al.   MOLECULAR PHARMACOLOGY

Arimoclomol at dosages up to 300 mg/day is well tolerated and safe in amyotrophic lateral sclerosis

(2008) Merit E. Cudkowicz et al.   MUSCLE & NERVE

Endoplasmic reticulum stress and induction of the unfolded protein response in human sporadic amyotrophic lateral sclerosis

(2008) Julie D. Atkin et al.   NEUROBIOLOGY OF DISEASE

Role of sarco/endoplasmic reticulum calcium content and calcium ATPase activity in the control of cell growth and proliferation

(2008) Larissa Lipskaia et al.   PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY

Amyotrophic Lateral Sclerosis: From Current Developments in the Laboratory to Clinical Implications

(2007) Mauro Cozzolino et al.   ANTIOXIDANTS & REDOX SIGNALING

Superoxide dismutase 1 mutants related to amyotrophic lateral sclerosis induce endoplasmic stress in neuro2a cells

(2007) Yeo Kyoung Oh et al.   JOURNAL OF NEUROCHEMISTRY