Impaired Autophagy and Defective Mitochondrial Function: Converging Paths on the Road to Motor Neuron Degeneration

Nuclear ULK1 promotes cell death in response to oxidative stress through PARP1

(2015) A Joshi et al.   CELL DEATH AND DIFFERENTIATION

MicroRNAs: Newcomers into the ALS Picture

(2015) Cinzia Volonte et al.   CNS & Neurological Disorders-Drug Targets

Neuronal Aggregates: Formation, Clearance, and Spreading

(2015) Junghyun Lim et al.   DEVELOPMENTAL CELL

The PINK1-PARKIN Mitochondrial Ubiquitylation Pathway Drives a Program of OPTN/NDP52 Recruitment and TBK1 Activation to Promote Mitophagy

(2015) Jin-Mi Heo et al.   MOLECULAR CELL

Mechanism of phospho-ubiquitin-induced PARKIN activation

(2015) Tobias Wauer et al.   NATURE

The ubiquitin kinase PINK1 recruits autophagy receptors to induce mitophagy

(2015) Michael Lazarou et al.   NATURE

Haploinsufficiency of TBK1 causes familial ALS and fronto-temporal dementia

(2015) Axel Freischmidt et al.   NATURE NEUROSCIENCE

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

(2015) M. Ikawa et al.   NEUROLOGY

Endolysosomal Deficits Augment Mitochondria Pathology in Spinal Motor Neurons of Asymptomatic fALS Mice

(2015) Yuxiang Xie et al.   NEURON

Exome sequencing in amyotrophic lateral sclerosis identifies risk genes and pathways

(2015) E. T. Cirulli et al.   SCIENCE

Reactive oxygen species trigger motoneuron death in non-cell-autonomous models of ALS through activation of c-Abl signaling

(2015) Fabiola Rojas et al.   Frontiers in Cellular Neuroscience

The role of TDP-43 in the pathogenesis of ALS and FTLD: Figure 1

(2015) Marco Baralle et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

Pathogenic Ubqln2 gains toxic properties to induce neuron death

(2014) Qinxue Wu et al.   ACTA NEUROPATHOLOGICA

Perturbations in intracellular Ca2+ handling in skeletal muscle in the G93A*SOD1 mouse model of amyotrophic lateral sclerosis

(2014) Eva R. Chin et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

UBQLN2mutation causing heterogeneous X-linked dominant neurodegeneration

(2014) Akl C. Fahed et al.   ANNALS OF NEUROLOGY

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

(2014) Kathryne Kirk et al.   ANNALS OF NEUROLOGY

Mutations in the CHCHD10 gene are a common cause of familial amyotrophic lateral sclerosis

(2014) Janel O. Johnson et al.   BRAIN

A mitochondrial origin for frontotemporal dementia and amyotrophic lateral sclerosis through CHCHD10 involvement

(2014) Sylvie Bannwarth et al.   BRAIN

Autosis and autophagic cell death: the dark side of autophagy

(2014) Y Liu et al.   CELL DEATH AND DIFFERENTIATION

AMBRA1 is able to induce mitophagy via LC3 binding, regardless of PARKIN and p62/SQSTM1

(2014) F Strappazzon et al.   CELL DEATH AND DIFFERENTIATION

Dysregulation of the proteasome increases the toxicity of ALS-linked mutant SOD1

(2014) Akira Kitamura et al.   GENES TO CELLS

C9ORF72, implicated in amytrophic lateral sclerosis and frontotemporal dementia, regulates endosomal trafficking

(2014) Manal A. Farg et al.   HUMAN MOLECULAR GENETICS

Ubiquilin 2: A component of the ubiquitin–proteasome system with an emerging role in neurodegeneration

(2014) Katharine Y. Zhang et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Mitochondrial trafficking and anchoring in neurons: New insight and implications

(2014) Zu-Hang Sheng   JOURNAL OF CELL BIOLOGY

Ubiquitin is phosphorylated by PINK1 to activate parkin

(2014) Fumika Koyano et al.   NATURE

Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models

(2014) Sami J Barmada et al.   Nature Chemical Biology

Regulated necrosis: the expanding network of non-apoptotic cell death pathways

(2014) Tom Vanden Berghe et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Screening of CHCHD10 in a French cohort confirms the involvement of this gene in frontotemporal dementia with amyotrophic lateral sclerosis patients

(2014) Annabelle Chaussenot et al.   NEUROBIOLOGY OF AGING

A Computational Model of Motor Neuron Degeneration

(2014) Gwendal Le Masson et al.   NEURON

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

(2014) Diane B. Re et al.   NEURON

Motor cortex glutathione deficit in ALS measured in vivo with the J-editing technique

(2014) N. Weiduschat et al.   NEUROSCIENCE LETTERS

Necroptosis

(2014) Andreas Linkermann et al.   NEW ENGLAND JOURNAL OF MEDICINE

Loss of Miro1-directed mitochondrial movement results in a novel murine model for neuron disease

(2014) T. T. Nguyen et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Optineurin is an autophagy receptor for damaged mitochondria in parkin-mediated mitophagy that is disrupted by an ALS-linked mutation

(2014) Yvette C. Wong et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Targeting autophagy in neurodegenerative diseases

(2014) René L. Vidal et al.   TRENDS IN PHARMACOLOGICAL SCIENCES

Mitochondrial Aging and Age-Related Dysfunction of Mitochondria

(2014) Dimitry A. Chistiakov et al.   Biomed Research International

Effects of mutations and deletions in the human optineurin gene

(2014) Sanja Turturro et al.   SpringerPlus

Protein aggregation in amyotrophic lateral sclerosis

(2013) Anna M. Blokhuis et al.   ACTA NEUROPATHOLOGICA

Mutations in SQSTM1 encoding p62 in amyotrophic lateral sclerosis: genetics and neuropathology

(2013) Elisa Teyssou et al.   ACTA NEUROPATHOLOGICA

Trehalose delays the progression of amyotrophic lateral sclerosis by enhancing autophagy in motoneurons

(2013) Karen Castillo et al.   Autophagy

Obatoclax (GX15-070) triggers necroptosis by promoting the assembly of the necrosome on autophagosomal membranes

(2013) F Basit et al.   CELL DEATH AND DIFFERENTIATION

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

(2013) Irina Lonskaya et al.   EMBO Molecular Medicine

Loss of iron triggers PINK1/Parkin-independent mitophagy

(2013) George F G Allen et al.   EMBO REPORTS

Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse models

(2013) Jordi Magrané et al.   HUMAN MOLECULAR GENETICS

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

Selective mitochondrial Ca2+uptake deficit in disease endstage vulnerable motoneurons of the SOD1G93Amouse model of amyotrophic lateral sclerosis

(2013) Andrea Fuchs et al.   JOURNAL OF PHYSIOLOGY-LONDON

Rapamycin increases survival in ALS mice lacking mature lymphocytes

(2013) Kim A Staats et al.   Molecular Neurodegeneration

Autophagy: A critical regulator of cellular metabolism and homeostasis

(2013) Stefan W. Ryter et al.   MOLECULES AND CELLS

ULK1 induces autophagy by phosphorylating Beclin-1 and activating VPS34 lipid kinase

(2013) Ryan C. Russell et al.   NATURE CELL BIOLOGY

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

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

Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis

(2013) Shuo-Chien Ling et al.   NEURON

Expanded GGGGCC repeat RNA associated with amyotrophic lateral sclerosis and frontotemporal dementia causes neurodegeneration

(2013) Z. Xu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Autosis is a Na+,K+-ATPase-regulated form of cell death triggered by autophagy-inducing peptides, starvation, and hypoxia-ischemia

(2013) Y. Liu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Autophagy in axonal and dendritic degeneration

(2013) Yi Yang et al.   TRENDS IN NEUROSCIENCES

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

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

Autophagy, neuron-specific degradation and neurodegeneration

(2012) Dong Wang et al.   Autophagy

Starvation-induced autophagy is regulated by mitochondrial reactive oxygen species leading to AMPK activation

(2012) Lin Li et al.   CELLULAR SIGNALLING

Mechanism and functions of membrane binding by the Atg5-Atg12/Atg16 complex during autophagosome formation

(2012) Julia Romanov et al.   EMBO JOURNAL

mTOR dysfunction contributes to vacuolar pathology and weakness in valosin-containing protein associated inclusion body myopathy

(2012) J. K. Ching et al.   HUMAN MOLECULAR GENETICS

PINK1- and Parkin-mediated mitophagy at a glance

(2012) Seok Min Jin et al.   JOURNAL OF CELL SCIENCE

Mitochondrial accumulation under oxidative stress is due to defects in autophagy

(2012) Cheng Luo et al.   JOURNAL OF CELLULAR BIOCHEMISTRY

Prion-like spread of protein aggregates in neurodegeneration: Figure 1.

(2012) Magdalini Polymenidou et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Mitochondrial Dynamics and Bioenergetic Dysfunction Is Associated with Synaptic Alterations in Mutant SOD1 Motor Neurons

(2012) J. Magrane et al.   JOURNAL OF NEUROSCIENCE

Mitochondrial outer-membrane protein FUNDC1 mediates hypoxia-induced mitophagy in mammalian cells

(2012) Lei Liu et al.   NATURE CELL BIOLOGY

Axonal transport deficits and degeneration can evolve independently in mouse models of amyotrophic lateral sclerosis

(2012) P. Marinkovic et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Autophagy activators rescue and alleviate pathogenesis of a mouse model with proteinopathies of the TAR DNA-binding protein 43

(2012) I.-F. Wang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

PINK1-mediated phosphorylation of the Parkin ubiquitin-like domain primes mitochondrial translocation of Parkin and regulates mitophagy

(2012) Kahori Shiba-Fukushima et al.   Scientific Reports

Discovery of Novel DENN Proteins: Implications for the Evolution of Eukaryotic Intracellular Membrane Structures and Human Disease

(2012) Dapeng Zhang et al.   Frontiers in Genetics

Vitamin E Intake and Risk of Amyotrophic Lateral Sclerosis: A Pooled Analysis of Data From 5 Prospective Cohort Studies

(2011) H. Wang et al.   AMERICAN JOURNAL OF EPIDEMIOLOGY

The Role of Atg Proteins in Autophagosome Formation

(2011) Noboru Mizushima et al.   Annual Review of Cell and Developmental Biology

Superoxide Dismutases: Role in Redox Signaling, Vascular Function, and Diseases

(2011) Tohru Fukai et al.   ANTIOXIDANTS & REDOX SIGNALING

Differential Involvement of Optineurin in Amyotrophic Lateral Sclerosis With or Without SOD1 Mutations

(2011) Han-Xiang Deng   ARCHIVES OF NEUROLOGY

SQSTM1 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

(2011) Faisal Fecto   ARCHIVES OF NEUROLOGY

Ambra1

(2011) Cindy Van Humbeeck et al.   Autophagy

Rapamycin treatment augments motor neuron degeneration in SOD1G93Amouse model of amyotrophic lateral sclerosis

(2011) Xiaojie Zhang et al.   Autophagy

Autophagy Dysregulation in Amyotrophic Lateral Sclerosis

(2011) Sheng Chen et al.   BRAIN PATHOLOGY

Upregulation of human autophagy-initiation kinase ULK1 by tumor suppressor p53 contributes to DNA-damage-induced cell death

(2011) W Gao et al.   CELL DEATH AND DIFFERENTIATION

Autophagy in the Cellular Energetic Balance

(2011) Rajat Singh et al.   Cell Metabolism

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

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

UBQLN2/P62 cellular recycling pathways in amyotrophic lateral sclerosis and frontotemporal dementia

(2011) Faisal Fecto et al.   MUSCLE & NERVE

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

(2011) Han-Xiang Deng et al.   NATURE

AMPK and mTOR regulate autophagy through direct phosphorylation of Ulk1

(2011) Joungmok Kim et al.   NATURE CELL BIOLOGY

VCP mutations in familial and sporadic amyotrophic lateral sclerosis

(2011) Max Koppers et al.   NEUROBIOLOGY OF AGING

Calcium dysregulation, mitochondrial pathology and protein aggregation in a culture model of amyotrophic lateral sclerosis: Mechanistic relationship and differential sensitivity to intervention

(2011) Miranda L. Tradewell et al.   NEUROBIOLOGY OF DISEASE

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

(2011) Mariely DeJesus-Hernandez et al.   NEURON

A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD

(2011) Alan E. Renton et al.   NEURON

Mitochondrial dysfunction in ALS

(2011) Mauro Cozzolino et al.   PROGRESS IN NEUROBIOLOGY

Autophagy Signaling Through Reactive Oxygen Species

(2010) Ju Huang et al.   ANTIOXIDANTS & REDOX SIGNALING

A Review of the Neural Mechanisms of Action and Clinical Efficiency of Riluzole in Treating Amyotrophic Lateral Sclerosis: What have we Learned in the Last Decade?

(2010) Mark C. Bellingham   CNS Neuroscience & Therapeutics

The role of the Atg1/ULK1 complex in autophagy regulation

(2010) Noboru Mizushima   CURRENT OPINION IN CELL BIOLOGY

Ubiquilin functions in autophagy and is degraded by chaperone-mediated autophagy

(2010) C. Rothenberg et al.   HUMAN MOLECULAR GENETICS

PINK1 cleavage at position A103 by the mitochondrial protease PARL

(2010) Emma Deas et al.   HUMAN MOLECULAR GENETICS

Mutations of optineurin in amyotrophic lateral sclerosis

(2010) Hirofumi Maruyama et al.   NATURE

Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS

(2010) Janel O. Johnson et al.   NEURON

The Mitochondrial Fusion-Promoting Factor Mitofusin Is a Substrate of the PINK1/Parkin Pathway

(2010) Angela C. Poole et al.   PLoS One

Deficits in axonal transport precede ALS symptoms in vivo

(2010) L. G. Bilsland et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ATM signals to TSC2 in the cytoplasm to regulate mTORC1 in response to ROS

(2010) A. Alexander 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

Phosphorylation of ULK1 (hATG1) by AMP-Activated Protein Kinase Connects Energy Sensing to Mitophagy

(2010) D. F. Egan et al.   SCIENCE

Regulation of autophagy by ROS: physiology and pathology

(2010) Ruth Scherz-Shouval et al.   TRENDS IN BIOCHEMICAL SCIENCES

Caspase-mediated cleavage of Beclin-1 inactivates Beclin-1-induced autophagy and enhances apoptosis by promoting the release of proapoptotic factors from mitochondria

(2010) E Wirawan et al.   Cell Death & Disease

Selective neuronal vulnerability to oxidative stress in the brain

(2010) Wang   Frontiers in Aging Neuroscience

PINK1 Is Selectively Stabilized on Impaired Mitochondria to Activate Parkin

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

Autophagy protects neuron from Aβ-induced cytotoxicity

(2009) Shih-Ya Hung et al.   Autophagy

Atg101, a novel mammalian autophagy protein interacting with Atg13

(2009) Nao Hosokawa et al.   Autophagy

Impairment of mitochondrial calcium handling in a mtSOD1 cell culture model of motoneuron disease

(2009) Manoj Jaiswal et al.   BMC NEUROSCIENCE

Tar DNA binding protein of 43 kDa (TDP-43), 14-3-3 proteins and copper/zinc superoxide dismutase (SOD1) interact to modulate NFL mRNA stability. Implications for altered RNA processing in amyotrophic lateral sclerosis (ALS)

(2009) Kathryn Volkening et al.   BRAIN RESEARCH

The Mechanism of Ca2+-Dependent Regulation of Kinesin-Mediated Mitochondrial Motility

(2009) Xinnan Wang et al.   CELL

PLIC proteins or ubiquilins regulate autophagy-dependent cell survival during nutrient starvation

(2009) Elsa-Noah N'Diaye et al.   EMBO REPORTS

The mitochondrial theory of aging: Insight from transgenic and knockout mouse models

(2009) Youngmok C. Jang et al.   EXPERIMENTAL GERONTOLOGY

TDP-43 Is Intrinsically Aggregation-prone, and Amyotrophic Lateral Sclerosis-linked Mutations Accelerate Aggregation and Increase Toxicity

(2009) Brian S. Johnson et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease

(2009) Jeong-Sun Ju et al.   JOURNAL OF CELL BIOLOGY

ULK-Atg13-FIP200 Complexes Mediate mTOR Signaling to the Autophagy Machinery

(2009) Chang Hwa Jung et al.   MOLECULAR BIOLOGY OF THE CELL

The Ψmdepolarization that accompanies mitochondrial Ca2+uptake is greater in mutant SOD1 than in wild-type mouse motor terminals

(2009) Khanh T. Nguyen et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

PINK1-dependent recruitment of Parkin to mitochondria in mitophagy

(2009) C. Vives-Bauza et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Inhibition of Autophagy Prevents Hippocampal Pyramidal Neuron Death after Hypoxic-Ischemic Injury

(2008) Masato Koike et al.   AMERICAN JOURNAL OF PATHOLOGY

A systematic review of antioxidant treatment for amyotrophic lateral sclerosis/motor neuron disease

(2008) Richard W. Orrell et al.   Amyotrophic Lateral Sclerosis

Role of Axonal Transport in Neurodegenerative Diseases

(2008) Kurt J. De Vos et al.   Annual Review of Neuroscience

Two German Kindreds With Familial Amyotrophic Lateral Sclerosis Due to TARDBP Mutations

(2008) Peter Kühnlein et al.   ARCHIVES OF NEUROLOGY

Docking of Axonal Mitochondria by Syntaphilin Controls Their Mobility and Affects Short-Term Facilitation

(2008) Jian-Sheng Kang et al.   CELL

FIP200, a ULK-interacting protein, is required for autophagosome formation in mammalian cells

(2008) Taichi Hara et al.   JOURNAL OF CELL BIOLOGY

TDP-43, the signature protein of FTLD-U, is a neuronal activity-responsive factor

(2008) I.-Fan Wang et al.   JOURNAL OF NEUROCHEMISTRY

Role of autophagy in G2019S-LRRK2-associated neurite shortening in differentiated SH-SY5Y cells

(2008) Edward D. Plowey et al.   JOURNAL OF NEUROCHEMISTRY

Cu/Zn Superoxide Dismutase Typical for Familial Amyotrophic Lateral Sclerosis Increases the Vulnerability of Mitochondria and Perturbs Ca2+ Homeostasis in SOD1G93A Mice

(2008) M. K. Jaiswal et al.   MOLECULAR PHARMACOLOGY

TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis

(2008) Edor Kabashi et al.   NATURE GENETICS

Low Ca2+ buffering in hypoglossal motoneurons of mutant SOD1 (G93A) mice

(2008) Friederike von Lewinski et al.   NEUROSCIENCE LETTERS

A yeast TDP-43 proteinopathy model: Exploring the molecular determinants of TDP-43 aggregation and cellular toxicity

(2008) B. S. Johnson et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The PINK1/Parkin pathway regulates mitochondrial morphology

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