Prevention of mitochondrial impairment by inhibition of protein phosphatase 1 activity in amyotrophic lateral sclerosis

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

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

C9ORF72-ALS/FTD-associated poly(GR) binds Atp5a1 and compromises mitochondrial function in vivo

(2019) So Yoen Choi et al.   NATURE NEUROSCIENCE

Spatiotemporal dynamics of molecular pathology in amyotrophic lateral sclerosis

(2019) Silas Maniatis et al.   SCIENCE

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

(2019) Peng Wang et al.   PLoS Genetics

Considerations for pharmacotherapy use in patients with amyotrophic lateral sclerosis: the earlier it starts, the better the results

(2019) Hideyuki Sawada   EXPERT OPINION ON PHARMACOTHERAPY

Inhibition of Drp1/Fis1 interaction slows progression of amyotrophic lateral sclerosis

(2018) Amit U Joshi et al.   EMBO Molecular Medicine

Differential toxicity of TDP-43 isoforms depends on their sub-mitochondrial localization in neuronal cells

(2018) Illari Salvatori et al.   JOURNAL OF NEUROCHEMISTRY

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

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

TDP-43 interacts with mitochondrial proteins critical for mitophagy and mitochondrial dynamics

(2018) Stephani A. Davis et al.   NEUROSCIENCE LETTERS

Abnormal RNA stability in amyotrophic lateral sclerosis

(2018) E. M. Tank et al.   Nature Communications

Drp1-Zip1 Interaction Regulates Mitochondrial Quality Surveillance System

(2018) Hyo Min Cho et al.   MOLECULAR CELL

The role of mitochondria in amyotrophic lateral sclerosis

(2017) Emma F. Smith et al.   NEUROSCIENCE LETTERS

Autophagy Dysregulation in ALS: When Protein Aggregates Get Out of Hand

(2017) Nandini Ramesh et al.   Frontiers in Molecular Neuroscience

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

(2016) Wenzhang Wang et al.   NATURE MEDICINE

Poly(GR) in C9ORF72 -Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons

(2016) Rodrigo Lopez-Gonzalez et al.   NEURON

Parkin Protects Against Misfolded SOD1 Toxicity by Promoting Its Aggresome Formation and Autophagic Clearance

(2015) Cheryl Yung et al.   MOLECULAR NEUROBIOLOGY

Translational profiling identifies a cascade of damage initiated in motor neurons and spreading to glia in mutant SOD1-mediated ALS

(2015) Shuying Sun et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Preventing proteostasis diseases by selective inhibition of a phosphatase regulatory subunit

(2015) I. Das et al.   SCIENCE

Increased in vivo glial activation in patients with amyotrophic lateral sclerosis: Assessed with [11C]-PBR28

(2015) Nicole R. Zürcher et al.   NeuroImage-Clinical

The regulation of mitochondrial dynamics

(2014) Suzanne Hoppins   CURRENT OPINION IN CELL BIOLOGY

The role of FUS gene variants in neurodegenerative diseases

(2014) Hao Deng et al.   Nature Reviews Neurology

Protein phosphatase 1 catalytic isoforms: specificity toward interacting proteins

(2014) Luís Korrodi-Gregório et al.   Translational Research

The LRRK2 inhibitor GSK2578215A induces protective autophagy in SH-SY5Y cells: involvement of Drp-1-mediated mitochondrial fission and mitochondrial-derived ROS signaling

(2014) S Saez-Atienzar et al.   Cell Death & Disease

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

Identification of protein phosphatase 1 as a regulator of the LRRK2 phosphorylation cycle

(2013) Evy Lobbestael et al.   BIOCHEMICAL JOURNAL

Mitochondrial Fusion and Fission Proteins Expression Dynamically Change in a Murine Model of Amyotrophic Lateral Sclerosis

(2013) Wentao Liu et al.   CURRENT NEUROVASCULAR RESEARCH

Synaptic NMDA receptor stimulation activates PP1 by inhibiting its phosphorylation by Cdk5

(2013) Hailong Hou et al.   JOURNAL OF CELL BIOLOGY

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

Amyloid-β oligomers induce tau-independent disruption of BDNF axonal transport via calcineurin activation in cultured hippocampal neurons

(2013) Elisa M. Ramser et al.   MOLECULAR BIOLOGY OF THE CELL

A Bcl-xL–Drp1 complex regulates synaptic vesicle membrane dynamics during endocytosis

(2013) Hongmei Li et al.   NATURE CELL BIOLOGY

Clinical Neurogenetics

(2013) Matthew B. Harms et al.   NEUROLOGIC CLINICS

An emerging role for misfolded wild-type SOD1 in sporadic ALS pathogenesis

(2013) Melissa S. Rotunno et al.   Frontiers in Cellular Neuroscience

Physiological and Pathological Significance of Dynamin-Related Protein 1 (Drp1)-Dependent Mitochondrial Fission in the Nervous System

(2013) Bongki Cho et al.   Experimental Neurobiology

Regulation of protein phosphatase 1 by intrinsically disordered proteins

(2012) Meng S. Choy et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

Clinico-pathological features in amyotrophic lateral sclerosis with expansions in C9ORF72

(2012) Johnathan Cooper-Knock et al.   BRAIN

Drp1-mediated mitochondrial dynamics and survival of developing chick motoneurons during the period of normal programmed cell death

(2012) So Yoen Choi et al.   FASEB JOURNAL

Structural basis for protein phosphatase 1 regulation and specificity

(2012) Wolfgang Peti et al.   FEBS Journal

Abnormal interaction between the mitochondrial fission protein Drp1 and hyperphosphorylated tau in Alzheimer's disease neurons: implications for mitochondrial dysfunction and neuronal damage

(2012) M. Manczak et al.   HUMAN MOLECULAR GENETICS

Cognitive and clinical characteristics of patients with amyotrophic lateral sclerosis carrying a C9orf72 repeat expansion: a population-based cohort study

(2012) Susan Byrne et al.   LANCET NEUROLOGY

Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study

(2012) Elisa Majounie et al.   LANCET NEUROLOGY

Mutant SOD1G93A triggers mitochondrial fragmentation in spinal cord motor neurons: Neuroprotection by SIRT3 and PGC-1α

(2012) Wenjun Song et al.   NEUROBIOLOGY OF DISEASE

Drug Screening for ALS Using Patient-Specific Induced Pluripotent Stem Cells

(2012) N. Egawa et al.   Science Translational Medicine

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

(2011) Evelyne Coussee et al.   CELL CALCIUM

Mitochondrial dysfunction in familial amyotrophic lateral sclerosis

(2011) Liesbeth Faes et al.   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

Parkin Ubiquitinates Drp1 for Proteasome-dependent Degradation

(2011) Hongxia Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Selective Neuronal Vulnerability in Neurodegenerative Diseases: from Stressor Thresholds to Degeneration

(2011) Smita Saxena et al.   NEURON

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

(2011) Mariely DeJesus-Hernandez et al.   NEURON

Impairing the Mitochondrial Fission and Fusion Balance: A New Mechanism of Neurodegeneration

(2010) Andrew B. Knott et al.   Annals of the New York Academy of Sciences

Modification of Superoxide Dismutase 1 (SOD1) Properties by a GFP Tag – Implications for Research into Amyotrophic Lateral Sclerosis (ALS)

(2010) James C. Stevens et al.   PLoS One

Amyotrophic lateral sclerosis

(2009) Lokesh C Wijesekera et al.   Orphanet Journal of Rare Diseases

Mice with Mitochondrial Complex I Deficiency Develop a Fatal Encephalomyopathy

(2008) Shane E. Kruse et al.   Cell Metabolism

Eukaryotic complex I: functional diversity and experimental systems to unravel the assembly process

(2008) Claire Remacle et al.   MOLECULAR GENETICS AND GENOMICS