CuATSM effectively ameliorates ALS patient astrocyte‐mediated motor neuron toxicity in human in vitro models of amyotrophic lateral sclerosis

Uncoupling proteins as a therapeutic target for the development of new era drugs against neurodegenerative disorder

(2022) Rahul Kumar et al.   BIOMEDICINE & PHARMACOTHERAPY

Searching Far and Genome-Wide: The Relevance of Association Studies in Amyotrophic Lateral Sclerosis

(2021) Kelly A. Rich et al.   Frontiers in Neuroscience

In vitro Modeling for Neurological Diseases using Direct Conversion from Fibroblasts to Neuronal Progenitor Cells and Differentiation into Astrocytes

(2021) Cassandra N. Dennys et al.   Jove-Journal of Visualized Experiments

Amyotrophic lateral sclerosis: a clinical review

(2020) P. Masrori et al.   EUROPEAN JOURNAL OF NEUROLOGY

Genetic Testing for Amyotrophic Lateral Sclerosis and Frontotemporal Dementia

(2020) Jennifer Roggenbuck et al.   CLINICS IN LABORATORY MEDICINE

Astrocyte adenosine deaminase loss increases motor neuron toxicity in amyotrophic lateral sclerosis

(2019) Scott P Allen et al.   BRAIN

Peroxynitrite supports a metabolic reprogramming in merlin-deficient Schwann cells and promotes cell survival

(2019) Jeanine C. Pestoni et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Integrative multi-omic analysis identifies new drivers and pathways in molecularly distinct subtypes of ALS

(2019) Giovanna Morello et al.   Scientific Reports

Neurological disorders and mitochondria

(2019) J.V. Cabral-Costa et al.   MOLECULAR ASPECTS OF MEDICINE

Neuroprotective effect of CuATSM on neurotoxin-induced motor neuron loss in an ALS mouse model

(2019) Michael T.H. Kuo et al.   NEUROBIOLOGY OF DISEASE

Modeling Protein Aggregation and the Heat Shock Response in ALS iPSC-Derived Motor Neurons

(2018) Emily R. Seminary et al.   Frontiers in Neuroscience

Modeling sporadic ALS in iPSC-derived motor neurons identifies a potential therapeutic agent

(2018) Koki Fujimori et al.   NATURE MEDICINE

Wildtype motoneurons, ALS-Linked SOD1 mutation and glutamate profoundly modify astrocyte metabolism and lactate shuttling

(2017) Blandine Madji Hounoum et al.   GLIA

Safety and efficacy of edaravone in well defined patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled trial

(2017) Koji Abe et al.   LANCET NEUROLOGY

High content analysis in amyotrophic lateral sclerosis

(2017) Federica Rinaldi et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

The Role of Metal Binding in the Amyotrophic Lateral Sclerosis-Related Aggregation of Copper-Zinc Superoxide Dismutase

(2017) et al.   MOLECULES

Amyotrophic Lateral Sclerosis

(2017) Robert H. Brown et al.   NEW ENGLAND JOURNAL OF MEDICINE

Edaravone and its clinical development for amyotrophic lateral sclerosis

(2017) Koji Takei et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

CuII(atsm) improves the neurological phenotype and survival of SOD1G93A mice and selectively increases enzymatically active SOD1 in the spinal cord

(2017) James B. Hilton et al.   Scientific Reports

Mitochondrial generation of superoxide and hydrogen peroxide as the source of mitochondrial redox signaling

(2016) Martin D. Brand   FREE RADICAL BIOLOGY AND MEDICINE

The Genetic Variability of UCP4 Affects the Individual Susceptibility to Late-Onset Alzheimer’s Disease and Modifies the Disease’s Risk in APOE-ɛ4 Carriers

(2016) Alberto Montesanto et al.   JOURNAL OF ALZHEIMERS DISEASE

Uncoupling protein 2 in the glial response to stress: implications for neuroprotection

(2016) ColinJ Barnstable et al.   Neural Regeneration Research

Copper delivery to the CNS by CuATSM effectively treats motor neuron disease in SODG93A mice co-expressing the Copper-Chaperone-for-SOD

(2016) Jared R. Williams et al.   NEUROBIOLOGY OF DISEASE

Increased oxidative stress is related to disease severity in the ALS motor cortex: A PET study

(2015) M. Ikawa et al.   NEUROLOGY

Resveratrol Preconditioning Protects Against Cerebral Ischemic Injury via Nuclear Erythroid 2–Related Factor 2

(2015) Srinivasan V. Narayanan et al.   STROKE

Control of Mitochondrial pH by Uncoupling Protein 4 in Astrocytes Promotes Neuronal Survival

(2014) Hélène Perreten Lambert et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Oral Treatment with CuII(atsm) Increases Mutant SOD1 In Vivo but Protects Motor Neurons and Improves the Phenotype of a Transgenic Mouse Model of Amyotrophic Lateral Sclerosis

(2014) B. R. Roberts et al.   JOURNAL OF NEUROSCIENCE

Necroptosis Drives Motor Neuron Death in Models of Both Sporadic and Familial ALS

(2014) Diane B. Re et al.   NEURON

Confirmatory double-blind, parallel-group, placebo-controlled study of efficacy and safety of edaravone (MCI-186) in amyotrophic lateral sclerosis patients

(2014) Koji Abe et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Mutant Copper-Zinc Superoxide Dismutase (SOD1) Induces Protein Secretion Pathway Alterations and Exosome Release in Astrocytes

(2013) Manuela Basso et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Direct conversion of patient fibroblasts demonstrates non-cell autonomous toxicity of astrocytes to motor neurons in familial and sporadic ALS

(2013) K. Meyer et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The hypoxia imaging agent Cu II (atsm) is neuroprotective and improves motor and cognitive functions in multiple animal models of Parkinson’s disease

(2012) Lin W. Hung et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Peroxisome proliferator-activated receptors γ/mitochondrial uncoupling protein 2 signaling protects against seizure-induced neuronal cell death in the hippocampus following experimental status epilepticus

(2012) Yao-Chung Chuang et al.   Journal of Neuroinflammation

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

(2011) Yasuyuki Honjo et al.   Amyotrophic Lateral Sclerosis

Dysregulation of astrocyte–motoneuron cross-talk in mutant superoxide dismutase 1-related amyotrophic lateral sclerosis

(2011) Laura Ferraiuolo et al.   BRAIN

Lanosterol induces mitochondrial uncoupling and protects dopaminergic neurons from cell death in a model for Parkinson's disease

(2011) L Lim et al.   CELL DEATH AND DIFFERENTIATION

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

Astrocytes from familial and sporadic ALS patients are toxic to motor neurons

(2011) Amanda M Haidet-Phillips et al.   NATURE BIOTECHNOLOGY

Evaluation of striatal oxidative stress in patients with Parkinson's disease using [62Cu]ATSM PET

(2011) Masamichi Ikawa et al.   NUCLEAR MEDICINE AND BIOLOGY

Radiolabeled Cu-ATSM as a novel indicator of overreduced intracellular state due to mitochondrial dysfunction: studies with mitochondrial DNA-less ρ0 cells and cybrids carrying MELAS mitochondrial DNA mutation

(2011) Yukie Yoshii et al.   NUCLEAR MEDICINE AND BIOLOGY

Astrocytes carrying the superoxide dismutase 1 (SOD1G93A) mutation induce wild-type motor neuron degeneration in vivo

(2011) S. T. Papadeas et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

An impaired mitochondrial electron transport chain increases retention of the hypoxia imaging agent diacetylbis(4-methylthiosemicarbazonato)copperII

(2011) P. S. Donnelly et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Deletion of Mitochondrial Uncoupling Protein-2 Increases Ischemic Brain Damage after Transient Focal Ischemia by Altering Gene Expression Patterns and Enhancing Inflammatory Cytokines

(2010) Bryan A Haines et al.   JOURNAL OF CEREBRAL BLOOD FLOW AND METABOLISM

Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS

(2010) Daryl A Bosco et al.   NATURE NEUROSCIENCE

Responders to ChEI Treatment of Alzheimers Disease Show Restitution of Normal Regional Cortical Activation

(2009) Annalena Venneri et al.   Current Alzheimer Research

A Genome-Wide Screen for Spatially Restricted Expression Patterns Identifies Transcription Factors That Regulate Glial Development

(2009) H. Fu et al.   JOURNAL OF NEUROSCIENCE

PET imaging of redox and energy states in stroke-like episodes of MELAS

(2009) Masamichi Ikawa et al.   MITOCHONDRION

TDP-43 is consistently co-localized with ubiquitinated inclusions in sporadic and Guam amyotrophic lateral sclerosis but not in familial amyotrophic lateral sclerosis with and without SOD1 mutations

(2009) Satomi Maekawa et al.   NEUROPATHOLOGY

Mitochondrial Uncoupling Protein-2 (UCP2) Mediates Leptin Protection Against MPP+ Toxicity in Neuronal Cells

(2009) Philip Wing-Lok Ho et al.   NEUROTOXICITY RESEARCH

Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis

(2008) Koji Yamanaka et al.   NATURE NEUROSCIENCE

Mitochondria in Neuroplasticity and Neurological Disorders

(2008) Mark P. Mattson et al.   NEURON