Proteinopathies and OXPHOS dysfunction in neurodegenerative diseases

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

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

(2017) Hibiki Kawamata et al.   Molecular Neurodegeneration

Persistence of the mitochondrial permeability transition in the absence of subunit c of human ATP synthase

(2017) Jiuya He et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

TDP-43 stabilises the processing intermediates of mitochondrial transcripts

(2017) Keiichi Izumikawa et al.   Scientific Reports

V63 and N65 of overexpressed α-synuclein are involved in mitochondrial dysfunction

(2016) Huilin Zhang et al.   BRAIN RESEARCH

ALS/FTD‐associated FUS activates GSK‐3β to disrupt the VAPB–PTPIP51 interaction and ER–mitochondria associations

(2016) Radu Stoica et al.   EMBO REPORTS

Localization and Processing of the Amyloid-β Protein Precursor in Mitochondria-Associated Membranes

(2016) Dolores Del Prete et al.   JOURNAL OF ALZHEIMERS DISEASE

Mitofusin-2 knockdown increases ER-mitochondria contact and decreases amyloid β-peptide production

(2016) Nuno Santos Leal et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

Monomeric Alpha-Synuclein Exerts a Physiological Role on Brain ATP Synthase

(2016) M. H. R. Ludtmann et al.   JOURNAL OF NEUROSCIENCE

Glutamate oxidation in astrocytes: Roles of glutamate dehydrogenase and aminotransferases

(2016) Mary C. McKenna et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Amyloid β-peptides interfere with mitochondrial preprotein import competence by a coaggregation process

(2016) Giovanna Cenini et al.   MOLECULAR BIOLOGY OF THE CELL

Mitochondrial Proteome Changes Correlating with β-Amyloid Accumulation

(2016) Katalin Völgyi et al.   MOLECULAR NEUROBIOLOGY

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

(2016) Wenzhang Wang et al.   NATURE MEDICINE

Synaptosomal Mitochondrial Dysfunction in 5xFAD Mouse Model of Alzheimer's Disease

(2016) Lu Wang et al.   PLoS One

α-Synuclein binds to TOM20 and inhibits mitochondrial protein import in Parkinson’s disease

(2016) Roberto Di Maio et al.   Science Translational Medicine

Deregulation of mitochondrial F1FO-ATP synthase via OSCP in Alzheimer’s disease

(2016) Simon J. Beck et al.   Nature Communications

Hexokinase I N-terminal based peptide prevents the VDAC1-SOD1 G93A interaction and re-establishes ALS cell viability

(2016) Andrea Magrì et al.   Scientific Reports

Early and gender-specific differences in spinal cord mitochondrial function and oxidative stress markers in a mouse model of ALS

(2016) Daniel Cacabelos et al.   Acta Neuropathologica Communications

ALS-linked misfolded SOD1 species have divergent impacts on mitochondria

(2016) Sarah Pickles et al.   Acta Neuropathologica Communications

Gene-specific mitochondria dysfunctions in human TARDBP and C9ORF72 fibroblasts

(2016) Elisa Onesto et al.   Acta Neuropathologica Communications

Structure and function of the mitochondrial calcium uniporter complex

(2015) Diego De Stefani et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Defective PITRM1 mitochondrial peptidase is associated with A  amyloidotic neurodegeneration

(2015) D. Brunetti et al.   EMBO Molecular Medicine

Mitochondrial ATP synthase activity is impaired by suppressedO-GlcNAcylation in Alzheimer's disease

(2015) Moon-Yong Cha et al.   HUMAN MOLECULAR GENETICS

Convergence of Parkin, PINK1, and α-Synuclein on Stress-induced Mitochondrial Morphological Remodeling

(2015) Kristi L. Norris et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

α-Synuclein Shows High Affinity Interaction with Voltage-dependent Anion Channel, Suggesting Mechanisms of Mitochondrial Regulation and Toxicity in Parkinson Disease

(2015) Tatiana K. Rostovtseva et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mitochondrial dysfunction in a transgenic mouse model expressing human amyloid precursor protein (APP) with the Arctic mutation

(2015) Annica Rönnbäck et al.   JOURNAL OF NEUROCHEMISTRY

A53T Human  -Synuclein Overexpression in Transgenic Mice Induces Pervasive Mitochondria Macroautophagy Defects Preceding Dopamine Neuron Degeneration

(2015) L. Chen et al.   JOURNAL OF NEUROSCIENCE

Genetic reduction of mitochondrial complex I function does not lead to loss of dopamine neurons in vivo

(2015) Hyung-Wook Kim et al.   NEUROBIOLOGY OF AGING

Macrophage Migration Inhibitory Factor as a Chaperone Inhibiting Accumulation of Misfolded SOD1

(2015) Adrian Israelson et al.   NEURON

The Roles of PINK1, Parkin, and Mitochondrial Fidelity in Parkinson’s Disease

(2015) Alicia M. Pickrell et al.   NEURON

MitoCarta2.0: an updated inventory of mammalian mitochondrial proteins

(2015) Sarah E. Calvo et al.   NUCLEIC ACIDS RESEARCH

α-Synuclein amino terminus regulates mitochondrial membrane permeability

(2014) Jiamei Shen et al.   BRAIN RESEARCH

Amyloid-β Peptide Induces Mitochondrial Dysfunction by Inhibition of Preprotein Maturation

(2014) Dirk Mossmann et al.   Cell Metabolism

Alpha-Synuclein Binds to the Inner Membrane of Mitochondria in an α-Helical Conformation

(2014) Marta Robotta et al.   CHEMBIOCHEM

Soluble, Prefibrillar α-Synuclein Oligomers Promote Complex I-dependent, Ca2+-induced Mitochondrial Dysfunction

(2014) Eric S. Luth et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

 -Synuclein Is Localized to Mitochondria-Associated ER Membranes

(2014) C. Guardia-Laguarta et al.   JOURNAL OF NEUROSCIENCE

Amyloid-Beta Disrupts Calcium and Redox Homeostasis in Brain Endothelial Cells

(2014) Ana Catarina R. G. Fonseca et al.   MOLECULAR NEUROBIOLOGY

Region specific mitochondrial impairment in mice with widespread overexpression of alpha-synuclein

(2014) Sudhakar Raja Subramaniam et al.   NEUROBIOLOGY OF DISEASE

Human A53T α-Synuclein Causes Reversible Deficits in Mitochondrial Function and Dynamics in Primary Mouse Cortical Neurons

(2014) Li Li et al.   PLoS One

An uncoupling channel within the c-subunit ring of the F1FO ATP synthase is the mitochondrial permeability transition pore

(2014) K. N. Alavian et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ER–mitochondria associations are regulated by the VAPB–PTPIP51 interaction and are disrupted by ALS/FTD-associated TDP-43

(2014) Radu Stoica et al.   Nature Communications

Mitochondrial membrane permeabilisation by amyloid aggregates and protection by polyphenols

(2013) Angelique Camilleri et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES

Mia40 and MINOS act in parallel with Ccs1 in the biogenesis of mitochondrial Sod1

(2013) Aksana Varabyova et al.   FEBS Journal

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

The Impact of Pathogenic Mitochondrial DNA Mutations on Substantia Nigra Neurons

(2013) A. Reeve et al.   JOURNAL OF NEUROSCIENCE

Mitochondrial DNA damage in a mouse model of Alzheimer's disease decreases amyloid beta plaque formation

(2013) Milena Pinto et al.   NEUROBIOLOGY OF AGING

VCP Is Essential for Mitochondrial Quality Control by PINK1/Parkin and this Function Is Impaired by VCP Mutations

(2013) Nam Chul Kim et al.   NEURON

α-Synuclein and Mitochondria: Partners in Crime?

(2013) Ken Nakamura   Neurotherapeutics

TOM40 Mediates Mitochondrial Dysfunction Induced by α-Synuclein Accumulation in Parkinson’s Disease

(2013) Andreas Bender et al.   PLoS One

Dimers of mitochondrial ATP synthase form the permeability transition pore

(2013) V. Giorgio et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Signaling the mitochondrial unfolded protein response

(2012) Mark W. Pellegrino et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Upregulated function of mitochondria-associated ER membranes in Alzheimer disease

(2012) Estela Area-Gomez et al.   EMBO JOURNAL

Cytochrome c oxidase dysfunction in oxidative stress

(2012) Satish Srinivasan et al.   FREE RADICAL BIOLOGY AND MEDICINE

Mitochondria-targeted catalase reduces abnormal APP processing, amyloid   production and BACE1 in a mouse model of Alzheimer's disease: implications for neuroprotection and lifespan extension

(2012) P. Mao et al.   HUMAN MOLECULAR GENETICS

α-Synuclein Controls Mitochondrial Calcium Homeostasis by Enhancing Endoplasmic Reticulum-Mitochondria Interactions

(2012) Tito Calì et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Amyloid-β Peptide Binds to Cytochrome C Oxidase Subunit 1

(2012) Luis Fernando Hernandez-Zimbron et al.   PLoS One

Assessing mitochondrial dysfunction in cells

(2011) Martin D. Brand et al.   BIOCHEMICAL JOURNAL

Matrix proteases in mitochondrial DNA function

(2011) Yuichi Matsushima et al.   Biochimica et Biophysica Acta-Gene Regulatory Mechanisms

Mitochondrial AAA proteases — Towards a molecular understanding of membrane-bound proteolytic machines

(2011) Florian Gerdes et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Regulating mitochondrial outer membrane proteins by ubiquitination and proteasomal degradation

(2011) Mariusz Karbowski et al.   CURRENT OPINION IN CELL BIOLOGY

VAPB interacts with the mitochondrial protein PTPIP51 to regulate calcium homeostasis

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

Ubiquitin-dependent mitochondrial protein degradation

(2011) Jin-Mi Heo et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Mutant A53T α-Synuclein Induces Neuronal Death by Increasing Mitochondrial Autophagy

(2011) Vinay Choubey et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Estrogen receptor mediates a distinct mitochondrial unfolded protein response

(2011) L. Papa et al.   JOURNAL OF CELL SCIENCE

In Vivo Pathogenic Role of Mutant SOD1 Localized in the Mitochondrial Intermembrane Space

(2011) A. Igoudjil et al.   JOURNAL OF NEUROSCIENCE

Inhibition of Amyloid-  (A ) Peptide-Binding Alcohol Dehydrogenase-A  Interaction Reduces A  Accumulation and Improves Mitochondrial Function in a Mouse Model of Alzheimer's Disease

(2011) J. Yao et al.   JOURNAL OF NEUROSCIENCE

Mia40-dependent oxidation of cysteines in domain I of Ccs1 controls its distribution between mitochondria and the cytosol

(2011) Christine Klöppel et al.   MOLECULAR BIOLOGY OF THE CELL

Mitochondrial Ccs1 contains a structural disulfide bond crucial for the import of this unconventional substrate by the disulfide relay system

(2011) Dominik P. Groß et al.   MOLECULAR BIOLOGY OF THE CELL

Mitochondrial Respiratory Chain Dysfunction in Muscle From Patients With Amyotrophic Lateral Sclerosis

(2010) Veronica Crugnola et al.   ARCHIVES OF NEUROLOGY

The Conserved Mitochondrial Twin Cx9C Protein Cmc2 Is a Cmc1 Homologue Essential for CytochromecOxidase Biogenesis

(2010) Darryl Horn et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Transgenic Overexpression of α-Synuclein and Not Its Related Pathology Associates with Complex I Inhibition

(2010) Virginie Loeb et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

(2010) Adrian Israelson et al.   NEURON

Mitochondrial alpha-synuclein accumulation impairs complex I function in dopaminergic neurons and results in increased mitophagy in vivo

(2010) Shankar J. Chinta et al.   NEUROSCIENCE LETTERS

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

PINK1 Is Selectively Stabilized on Impaired Mitochondria to Activate Parkin

(2010) Derek P. Narendra et al.   PLOS BIOLOGY

Mitochondrial Ubiquitin Ligase MITOL Ubiquitinates Mutant SOD1 and Attenuates Mutant SOD1-induced Reactive Oxygen Species Generation

(2009) Ryo Yonashiro et al.   MOLECULAR BIOLOGY OF THE CELL

Redox susceptibility of SOD1 mutants is associated with the differential response to CCS over-expression in vivo

(2009) Marjatta Son et al.   NEUROBIOLOGY OF DISEASE

Mitochondrial Disorders in the Nervous System

(2008) Salvatore DiMauro et al.   Annual Review of Neuroscience

Mitochondrial translocation of α-synuclein is promoted by intracellular acidification

(2008) Nelson B. Cole et al.   EXPERIMENTAL CELL RESEARCH

Different regulation of wild-type and mutant Cu,Zn superoxide dismutase localization in mammalian mitochondria

(2008) Hibiki Kawamata et al.   HUMAN MOLECULAR GENETICS

Mitochondrial Protein Quality Control by the Proteasome Involves Ubiquitination and the Protease Omi

(2008) Susanne Radke et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mitochondrial Import and Accumulation of α-Synuclein Impair Complex I in Human Dopaminergic Neuronal Cultures and Parkinson Disease Brain

(2008) Latha Devi et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Parkin is recruited selectively to impaired mitochondria and promotes their autophagy

(2008) Derek Narendra et al.   JOURNAL OF CELL BIOLOGY

The Disulfide Relay System of Mitochondria Is Required for the Biogenesis of Mitochondrial Ccs1 and Sod1

(2008) Silvia Reddehase et al.   JOURNAL OF MOLECULAR BIOLOGY

Optical Reporters for the Conformation of  -Synuclein Reveal a Specific Interaction with Mitochondria

(2008) K. Nakamura et al.   JOURNAL OF NEUROSCIENCE

Cyclophilin D deficiency attenuates mitochondrial and neuronal perturbation and ameliorates learning and memory in Alzheimer's disease

(2008) Heng Du et al.   NATURE MEDICINE

Genome-Wide and Functional Annotation of Human E3 Ubiquitin Ligases Identifies MULAN, a Mitochondrial E3 that Regulates the Organelle's Dynamics and Signaling

(2008) Wei Li et al.   PLoS One

Mitochondrial Ca2+ Overload Underlies Aβ Oligomers Neurotoxicity Providing an Unexpected Mechanism of Neuroprotection by NSAIDs

(2008) Sara Sanz-Blasco et al.   PLoS One

Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria

(2008) C. Vande Velde et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The amyloid  -peptide is imported into mitochondria via the TOM import machinery and localized to mitochondrial cristae

(2008) C. A. Hansson Petersen et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA