Mitochondrial dysfunction and neurodegenerative proteinopathies: mechanisms and prospects for therapeutic intervention

Oligomeric amyloid β preferentially targets neuronal and not glial mitochondrial-encoded mRNAs

(2018) Diego Mastroeni et al.   Alzheimers & Dementia

Synthesis and evaluation of 2-(3-arylureido)pyridines and 2-(3-arylureido)pyrazines as potential modulators of Aβ-induced mitochondrial dysfunction in Alzheimer's disease

(2018) Ahmed Elkamhawy et al.   EUROPEAN JOURNAL OF MEDICINAL CHEMISTRY

α-synuclein Induces Mitochondrial Dysfunction through Spectrin and the Actin Cytoskeleton

(2018) Dalila G. Ordonez et al.   NEURON

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

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

Cardiolipin exposure on the outer mitochondrial membrane modulates α-synuclein

(2018) Tammy Ryan et al.   Nature Communications

Decreased Proteolytic Activity of the Mitochondrial Amyloid-β Degrading Enzyme, PreP Peptidasome, in Alzheimer's Disease Brain Mitochondria

(2018) Nyosha Alikhani et al.   JOURNAL OF ALZHEIMERS DISEASE

Amyloid-β Peptides are Generated in Mitochondria-Associated Endoplasmic Reticulum Membranes

(2018) Bernadette Schreiner et al.   JOURNAL OF ALZHEIMERS DISEASE

Synthesis and evaluation of new pyridyl/pyrazinyl thiourea derivatives: Neuroprotection against amyloid-β-induced toxicity

(2017) Jung-eun Park et al.   EUROPEAN JOURNAL OF MEDICINAL CHEMISTRY

Interactions between Tau and Different Conformations of Tubulin: Implications for Tau Function and Mechanism

(2017) Aranda R. Duan et al.   JOURNAL OF MOLECULAR BIOLOGY

Extensive uptake of α-synuclein oligomers in astrocytes results in sustained intracellular deposits and mitochondrial damage

(2017) Veronica Lindström et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

PINK1 suppresses alpha-synuclein-induced neuronal injury: a novel mechanism in protein phosphatase 2A activation

(2017) Weiwei Yang et al.   Oncotarget

αSynuclein control of mitochondrial homeostasis in human-derived neurons is disrupted by mutations associated with Parkinson’s disease

(2017) Victorio Martin Pozo Devoto et al.   Scientific Reports

Antioxidants in Central Nervous System Diseases: Preclinical Promise and Translational Challenges

(2017) Chandrashekhar D. Kamat et al.   JOURNAL OF ALZHEIMERS DISEASE

Neuronal Calcium Signaling, Mitochondrial Dysfunction, and Alzheimer's Disease

(2017) Charlene Supnet et al.   JOURNAL OF ALZHEIMERS DISEASE

A NH2 Tau Fragment Targets Neuronal Mitochondria at AD Synapses: Possible Implications for Neurodegeneration

(2017) Giuseppina Amadoro et al.   JOURNAL OF ALZHEIMERS DISEASE

Mitochondrial permeability transition pore: sensitivity to opening and mechanistic dependence on substrate availability

(2017) Thomas Briston et al.   Scientific Reports

Quantification of active mitochondrial permeability transition pores using GNX-4975 inhibitor titrations provides insights into molecular identity

(2016) A. P. Richardson et al.   BIOCHEMICAL JOURNAL

Functional Impairment in Miro Degradation and Mitophagy Is a Shared Feature in Familial and Sporadic Parkinson’s Disease

(2016) Chung-Han Hsieh et al.   Cell Stem Cell

Pathogenic mechanisms of prion protein, amyloid-β and α-synuclein misfolding: the prion concept and neurotoxicity of protein oligomers

(2016) Cathryn L. Ugalde et al.   JOURNAL OF NEUROCHEMISTRY

Mitochondrial dysfunction in Parkinson's disease

(2016) Anindita Bose et al.   JOURNAL OF NEUROCHEMISTRY

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

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

Deubiquitinase Usp8 regulates α-synuclein clearance and modifies its toxicity in Lewy body disease

(2016) Zoi Alexopoulou et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

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

Identification of a Small Molecule Cyclophilin D Inhibitor for Rescuing Aβ-Mediated Mitochondrial Dysfunction

(2016) Koteswara Rao Valasani et al.   ACS Medicinal Chemistry Letters

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

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

Tau accumulation impairs mitophagy via increasing mitochondrial membrane potential and reducing mitochondrial Parkin

(2016) Yu Hu et al.   Oncotarget

Human wild-type full-length tau accumulation disrupts mitochondrial dynamics and the functions via increasing mitofusins

(2016) Xia-Chun Li et al.   Scientific Reports

Identification of ER-000444793, a Cyclophilin D-independent inhibitor of mitochondrial permeability transition, using a high-throughput screen in cryopreserved mitochondria

(2016) Thomas Briston et al.   Scientific Reports

Parkin and PINK1 Patient iPSC-Derived Midbrain Dopamine Neurons Exhibit Mitochondrial Dysfunction and α-Synuclein Accumulation

(2016) Sun Young Chung et al.   Stem Cell Reports

Deubiquitinating enzymes regulate PARK2-mediated mitophagy

(2015) Yuqing Wang et al.   Autophagy

N-Phenylbenzamides as Potent Inhibitors of the Mitochondrial Permeability Transition Pore

(2015) Sudeshna Roy et al.   ChemMedChem

Defective PITRM1 mitochondrial peptidase is associated with A  amyloidotic neurodegeneration

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

Proteinopathies, a core concept for understanding and ultimately treating degenerative disorders?

(2015) Thomas A. Bayer   EUROPEAN NEUROPSYCHOPHARMACOLOGY

NH2-truncated human tau induces deregulated mitophagy in neurons by aberrant recruitment of Parkin and UCHL-1: implications in Alzheimer's disease

(2015) V. Corsetti et al.   HUMAN MOLECULAR GENETICS

F-box protein 7 mutations promote protein aggregation in mitochondria and inhibit mitophagy

(2015) Zhi Dong Zhou et al.   HUMAN MOLECULAR GENETICS

Mitochondria: A Therapeutic Target for Parkinson’s Disease?

(2015) Yu Luo et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Mitochondrial impairment and oxidative stress compromise autophagosomal degradation of α-synuclein in oligodendroglial cells

(2015) Katharina Pukaß 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

USP30: A new promising target for Parkinson's disease?

(2015) S. Thobois   MOVEMENT DISORDERS

USP30 and parkin homeostatically regulate atypical ubiquitin chains on mitochondria

(2015) Christian N. Cunningham et al.   NATURE CELL BIOLOGY

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

(2015) Alicia M. Pickrell et al.   NEURON

α-Synuclein amino terminus regulates mitochondrial membrane permeability

(2014) Jiamei Shen et al.   BRAIN RESEARCH

A small natural molecule promotes mitochondrial fusion through inhibition of the deubiquitinase USP30

(2014) Wen Yue et al.   CELL RESEARCH

Loss of the m-AAA protease subunit AFG3L2 causes mitochondrial transport defects and tau hyperphosphorylation

(2014) A. K. Kondadi et al.   EMBO JOURNAL

USP8 regulates mitophagy by removing K6-linked ubiquitin conjugates from parkin

(2014) T. M. Durcan et al.   EMBO JOURNAL

The mitochondria-targeted antioxidant MitoQ extends lifespan and improves healthspan of a transgenic Caenorhabditis elegans model of Alzheimer disease

(2014) Li Fang Ng et al.   FREE RADICAL BIOLOGY AND MEDICINE

Neuroprotective effects of the mitochondria-targeted antioxidant MitoQ in a model of inherited amyotrophic lateral sclerosis

(2014) Ernesto Miquel et al.   FREE RADICAL BIOLOGY AND MEDICINE

Potentiation of neurotoxicity in double-mutant mice with Pink1 ablation and A53T-SNCA overexpression

(2014) Suzana Gispert et al.   HUMAN MOLECULAR GENETICS

Parkin-mediated reduction of nuclear and soluble TDP-43 reverses behavioral decline in symptomatic mice

(2014) Chen Wenqiang et al.   HUMAN MOLECULAR GENETICS

The deubiquitinase USP15 antagonizes Parkin-mediated mitochondrial ubiquitination and mitophagy

(2014) Tom Cornelissen et al.   HUMAN MOLECULAR GENETICS

Cinnamic Anilides as New Mitochondrial Permeability Transition Pore Inhibitors Endowed with Ischemia-Reperfusion Injury Protective Effect in Vivo

(2014) Daniele Fancelli et al.   JOURNAL OF MEDICINAL CHEMISTRY

 -Synuclein Is Localized to Mitochondria-Associated ER Membranes

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

The mitochondrial deubiquitinase USP30 opposes parkin-mediated mitophagy

(2014) Baris Bingol et al.   NATURE

GNX-4728, a novel small molecule drug inhibitor of mitochondrial permeability transition, is therapeutic in a mouse model of amyotrophic lateral sclerosis

(2014) Lee J. Martin et al.   Frontiers in Cellular Neuroscience

Mitochondrial dysfunction in amyotrophic lateral sclerosis - a valid pharmacological target?

(2013) H Muyderman et al.   BRITISH JOURNAL OF PHARMACOLOGY

Mitochondria and Quality Control Defects in a Mouse Model of Gaucher Disease—Links to Parkinson’s Disease

(2013) Laura D. Osellame et al.   Cell Metabolism

Tyrosine kinase inhibition increases functional parkin-Beclin-1 interaction and enhances amyloid clearance and cognitive performance

(2013) Irina Lonskaya et al.   EMBO Molecular Medicine

The ALS disease-associated mutant TDP-43 impairs mitochondrial dynamics and function in motor neurons

(2013) Wenzhang Wang et al.   HUMAN MOLECULAR GENETICS

Parkin overexpression ameliorates hippocampal long-term potentiation and  -amyloid load in an Alzheimer's disease mouse model

(2013) X. Hong et al.   HUMAN MOLECULAR GENETICS

Down-regulation of Mortalin Exacerbates Aβ-mediated Mitochondrial Fragmentation and Dysfunction

(2013) So Jung Park et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Nilotinib-induced autophagic changes increase endogenous parkin level and ubiquitination, leading to amyloid clearance

(2013) Irina Lonskaya et al.   JOURNAL OF MOLECULAR MEDICINE-JMM

The Parkinson's disease–linked proteins Fbxo7 and Parkin interact to mediate mitophagy

(2013) Victoria S Burchell et al.   NATURE NEUROSCIENCE

AD-linked, toxic NH2 human tau affects the quality control of mitochondria in neurons

(2013) G. Amadoro et al.   NEUROBIOLOGY OF DISEASE

Cyclophilin D Deficiency Rescues Axonal Mitochondrial Transport in Alzheimer’s Neurons

(2013) Lan Guo 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

Role of PINK1 Binding to the TOM Complex and Alternate Intracellular Membranes in Recruitment and Activation of the E3 Ligase Parkin

(2012) Michael Lazarou et al.   DEVELOPMENTAL CELL

Synthesis and evaluation of oxime derivatives as modulators for amyloid beta-induced mitochondrial dysfunction

(2012) Young Seub Kim et al.   EUROPEAN JOURNAL OF MEDICINAL CHEMISTRY

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

Abnormal interaction of VDAC1 with amyloid beta and phosphorylated tau causes mitochondrial dysfunction in Alzheimer's disease

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

Tau Promotes Neurodegeneration via DRP1 Mislocalization In Vivo

(2012) Brian DuBoff et al.   NEURON

Loss of Axonal Mitochondria Promotes Tau-Mediated Neurodegeneration and Alzheimer's Disease–Related Tau Phosphorylation Via PAR-1

(2012) Kanae Iijima-Ando et al.   PLoS Genetics

Loss of Prohibitin Membrane Scaffolds Impairs Mitochondrial Architecture and Leads to Tau Hyperphosphorylation and Neurodegeneration

(2012) Carsten Merkwirth et al.   PLoS Genetics

Tau Accumulation Causes Mitochondrial Distribution Deficits in Neurons in a Mouse Model of Tauopathy and in Human Alzheimer's Disease Brain

(2011) Katherine J. Kopeikina et al.   AMERICAN JOURNAL OF PATHOLOGY

Mitochondria: The Common Upstream Driver of Amyloid-β and Tau Pathology in Alzheimers Disease

(2011) D. F.F. Silva et al.   Current Alzheimer Research

FTD and ALS: A Tale of Two Diseases

(2011) R. Ferrari et al.   Current Alzheimer Research

Parkin mediates beclin-dependent autophagic clearance of defective mitochondria and ubiquitinated A  in AD models

(2011) P. J. Khandelwal et al.   HUMAN MOLECULAR GENETICS

α-Synuclein overexpression impairs mitochondrial function by associating with adenylate translocator

(2011) Yuangang Zhu et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Direct Membrane Association Drives Mitochondrial Fission by the Parkinson Disease-associated Protein α-Synuclein

(2011) Ken Nakamura et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Mitochondria-Targeted Antioxidant MitoQ Prevents Loss of Spatial Memory Retention and Early Neuropathology in a Transgenic Mouse Model of Alzheimer's Disease

(2011) M. J. McManus et al.   JOURNAL OF NEUROSCIENCE

Interaction between NH2-tau fragment and Aβ in Alzheimer's disease mitochondria contributes to the synaptic deterioration

(2011) Giuseppina Amadoro et al.   NEUROBIOLOGY OF AGING

 -Synuclein fate is determined by USP9X-regulated monoubiquitination

(2011) R. Rott et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

FUS Transgenic Rats Develop the Phenotypes of Amyotrophic Lateral Sclerosis and Frontotemporal Lobar Degeneration

(2011) Cao Huang et al.   PLoS Genetics

Extensive FUS-Immunoreactive Pathology in Juvenile Amyotrophic Lateral Sclerosis with Basophilic Inclusions

(2010) Eric J. Huang et al.   BRAIN PATHOLOGY

Mortalin overexpression attenuates beta-amyloid-induced neurotoxicity in SH-SY5Y cells

(2010) Mingyue Qu et al.   BRAIN RESEARCH

The dysregulation of intracellular calcium in Alzheimer disease

(2010) Charlene Supnet et al.   CELL CALCIUM

Calcium-dependent mitochondrial function and dysfunction in neurons

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

Wild-Type Human TDP-43 Expression Causes TDP-43 Phosphorylation, Mitochondrial Aggregation, Motor Deficits, and Early Mortality in Transgenic Mice

(2010) Y.-F. Xu et al.   JOURNAL OF NEUROSCIENCE

TRO40303, a New Cardioprotective Compound, Inhibits Mitochondrial Permeability Transition

(2010) S. Schaller et al.   JOURNAL OF PHARMACOLOGY AND EXPERIMENTAL THERAPEUTICS

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

Phosphorylation by the c-Abl protein tyrosine kinase inhibits parkin's ubiquitination and protective function

(2010) H. S. Ko et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice

(2010) X. Shan et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The cyclophilin inhibitor Debio 025 normalizes mitochondrial function, muscle apoptosis and ultrastructural defects inCol6a1−/−myopathic mice

(2009) T Tiepolo et al.   BRITISH JOURNAL OF PHARMACOLOGY

Amyloid-beta Leads to Impaired Cellular Respiration, Energy Production and Mitochondrial Electron Chain Complex Activities in Human Neuroblastoma Cells

(2009) V. Rhein et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

Parkin promotes intracellular A 1-42 clearance

(2009) M. P. Burns et al.   HUMAN MOLECULAR GENETICS

Cyclophilin D Modulates Mitochondrial F0F1-ATP Synthase by Interacting with the Lateral Stalk of the Complex

(2009) Valentina Giorgio et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Differential effect of three-repeat and four-repeat tau on mitochondrial axonal transport

(2009) Will Stoothoff et al.   JOURNAL OF NEUROCHEMISTRY

Deubiquitinase USP9X stabilizes MCL1 and promotes tumour cell survival

(2009) Martin Schwickart et al.   NATURE

Cyclophilin D deficiency improves mitochondrial function and learning/memory in aging Alzheimer disease mouse model

(2009) Heng Du et al.   NEUROBIOLOGY OF AGING

Amyloid-  and tau synergistically impair the oxidative phosphorylation system in triple transgenic Alzheimer's disease mice

(2009) V. Rhein et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Regulation of Mitochondrial Morphology by USP30, a Deubiquitinating Enzyme Present in the Mitochondrial Outer Membrane

(2008) Nobuhiro Nakamura et al.   MOLECULAR BIOLOGY OF THE CELL

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

(2008) Heng Du et al.   NATURE MEDICINE

Study of subcellular localization and proteolysis of ataxin-3

(2008) Chiara Pozzi et al.   NEUROBIOLOGY OF DISEASE

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