Neuronal mitochondrial dysfunction in sporadic amyotrophic lateral sclerosis is developmentally regulated

RNA Dysregulation in Amyotrophic Lateral Sclerosis

(2019) Zoe Butti et al.   Frontiers in Genetics

Mitochondrial defect in muscle precedes neuromuscular junction degeneration and motor neuron death in CHCHD10S59L/+ mouse

(2019) Emmanuelle C. Genin et al.   ACTA NEUROPATHOLOGICA

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

(2019) Peng Wang et al.   PLoS Genetics

Mutant huntingtin disrupts mitochondrial proteostasis by interacting with TIM23

(2019) Svitlana Yablonska et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Energy metabolism in ALS: an underappreciated opportunity?

(2018) Tijs Vandoorne et al.   ACTA NEUROPATHOLOGICA

Discrete mitochondrial aberrations in the spinal cord of sporadic ALS patients

(2018) Vedad Delic et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Characterization of the Mitochondrial Aerobic Metabolism in the Pre- and Perisynaptic Districts of the SOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis

(2018) Silvia Ravera et al.   MOLECULAR NEUROBIOLOGY

The limited application of stem cells in medicine: a review

(2018) Jordan Poulos   Stem Cell Research & Therapy

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

(2018) Koki Fujimori et al.   NATURE MEDICINE

Mitochondria, ER, and nuclear membrane defects reveal early mechanisms for upper motor neuron vulnerability with respect to TDP-43 pathology

(2018) Mukesh Gautam et al.   ACTA NEUROPATHOLOGICA

Distinction of sporadic and familial forms of ALS based on mitochondrial characteristics

(2018) Jarosław Walczak et al.   FASEB JOURNAL

Defective mitochondrial protein import contributes to complex I-induced mitochondrial dysfunction and neurodegeneration in Parkinson’s disease

(2018) Sandra Franco-Iborra et al.   Cell Death & Disease

ALS Pathogenesis and Therapeutic Approaches: The Role of Mesenchymal Stem Cells and Extracellular Vesicles

(2017) Roberta Bonafede et al.   Frontiers in Cellular Neuroscience

Metabolic Dysfunctions in Amyotrophic Lateral Sclerosis Pathogenesis and Potential Metabolic Treatments

(2017) Tesfaye W. Tefera et al.   Frontiers in Neuroscience

Protein Import by the Mitochondrial Presequence Translocase in the Absence of a Membrane Potential

(2016) Uma Turakhiya et al.   JOURNAL OF MOLECULAR BIOLOGY

Trehalose upregulates progranulin expression in human and mouse models of GRN haploinsufficiency: a novel therapeutic lead to treat frontotemporal dementia

(2016) Christopher J. Holler et al.   Molecular Neurodegeneration

Modeling ALS with motor neurons derived from human induced pluripotent stem cells

(2016) Samuel Sances et al.   NATURE NEUROSCIENCE

Pathophysiological and diagnostic implications of cortical dysfunction in ALS

(2016) Nimeshan Geevasinga et al.   Nature Reviews Neurology

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

C9orf72 Hexanucleotide Expansions Are Associated with Altered Endoplasmic Reticulum Calcium Homeostasis and Stress Granule Formation in Induced Pluripotent Stem Cell-Derived Neurons from Patients with Amyotrophic Lateral Sclerosis and Frontotemporal Demen

(2016) Ruxandra Dafinca et al.   STEM CELLS

Metabolic reprogramming during neuronal differentiation from aerobic glycolysis to neuronal oxidative phosphorylation

(2016) Xinde Zheng et al.   eLife

Metabolomic Analysis Reveals Increased Aerobic Glycolysis and Amino Acid Deficit in a Cellular Model of Amyotrophic Lateral Sclerosis

(2015) Gabriel N. Valbuena et al.   MOLECULAR NEUROBIOLOGY

Differentiation of Human Neural Stem Cells into Motor Neurons Stimulates Mitochondrial Biogenesis and Decreases Glycolytic Flux

(2015) Laura C. O'Brien et al.   STEM CELLS AND DEVELOPMENT

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

Mitochondrial dynamic abnormalities in amyotrophic lateral sclerosis

(2015) Zhen Jiang et al.   Translational Neurodegeneration

Bioenergetic markers in skin fibroblasts of sporadic amyotrophic lateral sclerosis and progressive lateral sclerosis patients

(2014) Kathryne Kirk et al.   ANNALS OF NEUROLOGY

Activity-Driven Local ATP Synthesis Is Required for Synaptic Function

(2014) Vidhya Rangaraju et al.   CELL

Pluripotent stem cell energy metabolism: an update

(2014) T. Teslaa et al.   EMBO JOURNAL

Single-Cell Imaging of Bioenergetic Responses to Neuronal Excitotoxicity and Oxygen and Glucose Deprivation

(2014) N. M. C. Connolly et al.   JOURNAL OF NEUROSCIENCE

Inhibition of mitochondrial protein import by mutant huntingtin

(2014) Hiroko Yano et al.   NATURE NEUROSCIENCE

The phenotypic variability of amyotrophic lateral sclerosis

(2014) Bart Swinnen et al.   Nature Reviews Neurology

Mitochondrial Oxidative Phosphorylation Transcriptome Alterations in Human Amyotrophic Lateral Sclerosis Spinal Cord and Blood

(2014) Amy C. Ladd et al.   NEUROMOLECULAR MEDICINE

Oxidative stress response elicited by mitochondrial dysfunction: Implication in the pathophysiology of aging

(2013) Chih-Hao Wang et al.   EXPERIMENTAL BIOLOGY AND MEDICINE

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

A cellular model for sporadic ALS using patient-derived induced pluripotent stem cells

(2013) Matthew F. Burkhardt et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

State of play in amyotrophic lateral sclerosis genetics

(2013) Alan E Renton et al.   NATURE NEUROSCIENCE

Superoxide dismutase 1 mutation in a cellular model of amyotrophic lateral sclerosis shifts energy generation from oxidative phosphorylation to glycolysis

(2013) Scott P. Allen et al.   NEUROBIOLOGY OF AGING

Overexpressed neuroglobin raises threshold for nitric oxide-induced impairment of mitochondrial respiratory activities and stress signaling in primary cortical neurons

(2013) Shilpee Singh et al.   NITRIC OXIDE-BIOLOGY AND CHEMISTRY

The Effect of SOD1 Mutation on Cellular Bioenergetic Profile and Viability in Response to Oxidative Stress and Influence of Mutation-Type

(2013) Katie Richardson et al.   PLoS One

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

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

Nuclear-encoded mitochondrial proteins and their role in cardioprotection

(2011) Kerstin Boengler et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Amyotrophic lateral sclerosis

(2011) Matthew C Kiernan et al.   LANCET

Energy Metabolism in Human Pluripotent Stem Cells and Their Differentiated Counterparts

(2011) Sandra Varum et al.   PLoS One

Energy metabolism in amyotrophic lateral sclerosis

(2010) Luc Dupuis et al.   LANCET NEUROLOGY

ALS-linked mutant superoxide dismutase 1 (SOD1) alters mitochondrial protein composition and decreases protein import

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

Mitochondrial Function, Morphology, and Axonal Transport in Amyotrophic Lateral Sclerosis

(2009) Jordi Magrané et al.   ANTIOXIDANTS & REDOX SIGNALING

Multiple pathways for mitochondrial protein traffic

(2009) Toshiya Endo et al.   BIOLOGICAL CHEMISTRY

Importing Mitochondrial Proteins: Machineries and Mechanisms

(2009) Agnieszka Chacinska et al.   CELL

Induced Pluripotent Stem Cells Generated from Patients with ALS Can Be Differentiated into Motor Neurons

(2008) J. T. Dimos et al.   SCIENCE