The Regulation of the Small Heat Shock Protein B8 in Misfolding Protein Diseases Causing Motoneuronal and Muscle Cell Death

Genetic Convergence Brings Clarity to the Enigmatic Red Line in ALS

(2019) Casey Cook et al.   NEURON

Cytoplasmic TDP-43 De-mixing Independent of Stress Granules Drives Inhibition of Nuclear Import, Loss of Nuclear TDP-43, and Cell Death

(2019) Fatima Gasset-Rosa et al.   NEURON

Chronic optogenetic induction of stress granules is cytotoxic and reveals the evolution of ALS-FTD pathology

(2019) Peipei Zhang et al.   eLife

Proteostasis and ALS: protocol for a phase II, randomised, double-blind, placebo-controlled, multicentre clinical trial for colchicine in ALS (Co-ALS)

(2019) Jessica Mandrioli et al.   BMJ Open

Unraveling the role of motoneuron autophagy in ALS

(2018) Vicente Valenzuela et al.   Autophagy

Pre-clinical symptoms of SBMA may not be androgen-dependent: implications from two SBMA mouse models

(2018) Youfen Xu et al.   HUMAN MOLECULAR GENETICS

Most mutations that cause spinocerebellar ataxia autosomal recessive type 16 (SCAR16) destabilize the protein quality-control E3 ligase CHIP

(2018) Adam J. Kanack et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Amyotrophic Lateral Sclerosis: An Update for 2018

(2018) Björn Oskarsson et al.   MAYO CLINIC PROCEEDINGS

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

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

Autophagy and mitophagy in ALS

(2018) Chantell S. Evans et al.   NEUROBIOLOGY OF DISEASE

Autophagy as a common pathway in amyotrophic lateral sclerosis

(2018) Dao K.H. Nguyen et al.   NEUROSCIENCE LETTERS

Tdp-25 Routing to Autophagy and Proteasome Ameliorates its Aggregation in Amyotrophic Lateral Sclerosis Target Cells

(2018) Maria Elena Cicardi et al.   Scientific Reports

Disrupted structure and aberrant function of CHIP mediates the loss of motor and cognitive function in preclinical models of SCAR16

(2018) Chang-he Shi et al.   PLoS Genetics

Trehalose induces autophagy via lysosomal-mediated TFEB activation in models of motoneuron degeneration

(2018) Paola Rusmini et al.   Autophagy

Heterozygous STUB1 mutation causes familial ataxia with cognitive affective syndrome (SCA48)

(2018) David Genis et al.   NEUROLOGY

Rapidly progressive amyotrophic lateral sclerosis is associated with microglial reactivity and small heat shock protein expression in reactive astrocytes

(2018) R. P. Gorter et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

HSPB1 mutations causing hereditary neuropathy in humans disrupt non-cell autonomous protection of motor neurons

(2017) Patrick L. Heilman et al.   EXPERIMENTAL NEUROLOGY

BAG3 Is a Modular, Scaffolding Protein that physically Links Heat Shock Protein 70 (Hsp70) to the Small Heat Shock Proteins

(2017) Jennifer N. Rauch et al.   JOURNAL OF MOLECULAR BIOLOGY

Biomolecular condensates: organizers of cellular biochemistry

(2017) Salman F. Banani et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

TIA1 Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Promote Phase Separation and Alter Stress Granule Dynamics

(2017) Ian R. Mackenzie et al.   NEURON

Lost in Transportation: Nucleocytoplasmic Transport Defects in ALS and Other Neurodegenerative Diseases

(2017) Hong Joo Kim et al.   NEURON

STUB1/CHIP mutations cause Gordon Holmes syndrome as part of a widespread multisystemic neurodegeneration: evidence from four novel mutations

(2017) Stefanie Nicole Hayer et al.   Orphanet Journal of Rare Diseases

The small heat shock protein B8 (HSPB8) modulates proliferation and migration of breast cancer cells

(2017) Margherita Piccolella et al.   Oncotarget

The Role of the Heat Shock Protein B8 (HSPB8) in Motoneuron Diseases

(2017) Paola Rusmini et al.   Frontiers in Molecular Neuroscience

Cellular Chaperones As Therapeutic Targets in ALS to Restore Protein Homeostasis and Improve Cellular Function

(2017) Bernadett Kalmar et al.   Frontiers in Molecular Neuroscience

The Hsp70/Hsp90 Chaperone Machinery in Neurodegenerative Diseases

(2017) Rachel E. Lackie et al.   Frontiers in Neuroscience

Glycolytic-to-oxidative fiber-type switch and mTOR signaling activation are early-onset features of SBMA muscle modified by high-fat diet

(2016) Anna Rocchi et al.   ACTA NEUROPATHOLOGICA

Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition)

(2016) Daniel J Klionsky et al.   Autophagy

C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles

(2016) Kyung-Ha Lee et al.   CELL

Estrogens, Neuroinflammation, and Neurodegeneration

(2016) Alessandro Villa et al.   ENDOCRINE REVIEWS

The chaperone HSPB8 reduces the accumulation of truncated TDP-43 species in cells and protects against TDP-43-mediated toxicity

(2016) Valeria Crippa et al.   HUMAN MOLECULAR GENETICS

Molecular Chaperones in the Pathogenesis of Amyotrophic Lateral Sclerosis: The Role of HSPB1

(2016) Simona Capponi et al.   HUMAN MUTATION

Defects in Neuromuscular Transmission May Underlie Motor Dysfunction in Spinal and Bulbar Muscular Atrophy

(2016) Youfen Xu et al.   JOURNAL OF NEUROSCIENCE

A Surveillance Function of the HSPB8-BAG3-HSP70 Chaperone Complex Ensures Stress Granule Integrity and Dynamism

(2016) Massimo Ganassi et al.   MOLECULAR CELL

Decoding ALS: from genes to mechanism

(2016) J. Paul Taylor et al.   NATURE

Breaking BAG: The Co-Chaperone BAG3 in Health and Disease

(2016) Christian Behl   TRENDS IN PHARMACOLOGICAL SCIENCES

Diagnostically important muscle pathology in DNAJB6 mutated LGMD1D

(2016) Satu Sandell et al.   Acta Neuropathologica Communications

Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD

(2016) Steven Boeynaems et al.   Scientific Reports

Transcriptional induction of the heat shock protein B8 mediates the clearance of misfolded proteins responsible for motor neuron diseases

(2016) Valeria Crippa et al.   Scientific Reports

Induction and adaptation of chaperone-assisted selective autophagy CASA in response to resistance exercise in human skeletal muscle

(2015) Anna Ulbricht et al.   Autophagy

Phase Separation by Low Complexity Domains Promotes Stress Granule Assembly and Drives Pathological Fibrillization

(2015) Amandine Molliex et al.   CELL

A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation

(2015) Avinash Patel et al.   CELL

Androgen-dependent loss of muscle BDNF mRNA in two mouse models of SBMA

(2015) Katherine Halievski et al.   EXPERIMENTAL NEUROLOGY

Silencing neuronal mutant androgen receptor in a mouse model of spinal and bulbar muscular atrophy

(2015) Kentaro Sahashi et al.   HUMAN MOLECULAR GENETICS

Chaperone mediated autophagy to the rescue: A new-fangled target for the treatment of neurodegenerative diseases

(2015) Maria Xilouri et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Treatment with Trehalose Prevents Behavioral and Neurochemical Deficits Produced in an AAV α-Synuclein Rat Model of Parkinson’s Disease

(2015) Qing He et al.   MOLECULAR NEUROBIOLOGY

GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport

(2015) Brian D. Freibaum et al.   NATURE

Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS

(2015) Ana Jovičić et al.   NATURE NEUROSCIENCE

Differences in protein quality control correlate with phenotype variability in 2 mouse models of familial amyotrophic lateral sclerosis

(2015) Marianna Marino et al.   NEUROBIOLOGY OF AGING

Mutations inHSPB8causing a new phenotype of distal myopathy and motor neuropathy

(2015) Roula Ghaoui et al.   NEUROLOGY

Trehalose decreases mutant SOD1 expression and alleviates motor deficiency in early but not end-stage amyotrophic lateral sclerosis in a SOD1-G93A mouse model

(2015) Y. Li et al.   NEUROSCIENCE

Cytoplasmic protein aggregates interfere with nucleocytoplasmic transport of protein and RNA

(2015) A. C. Woerner et al.   SCIENCE

UPR, autophagy, and mitochondria crosstalk underlies the ER stress response

(2015) Daniela Senft et al.   TRENDS IN BIOCHEMICAL SCIENCES

Aberrant Autophagic Response in The Muscle of A Knock-in Mouse Model of Spinal and Bulbar Muscular Atrophy

(2015) Paola Rusmini et al.   Scientific Reports

A Role for the Chaperone Complex BAG3-HSPB8 in Actin Dynamics, Spindle Orientation and Proper Chromosome Segregation during Mitosis

(2015) Margit Fuchs et al.   PLoS Genetics

Motoneuronal and muscle-selective removal of ALS-related misfolded proteins: Figure 1

(2015) Valeria Crippa et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

HspB8 and Bag3: A new chaperone complex targeting misfolded proteins to macroautophagy

(2014) Serena Carra et al.   Autophagy

BAG3 induces the sequestration of proteasomal clients into cytoplasmic puncta

(2014) Melania Minoia et al.   Autophagy

MTOR-independent, autophagic enhancer trehalose prolongs motor neuron survival and ameliorates the autophagic flux defect in a mouse model of amyotrophic lateral sclerosis

(2014) Xiaojie Zhang et al.   Autophagy

Autophagy in the physiology and pathology of the central nervous system

(2014) V Nikoletopoulou et al.   CELL DEATH AND DIFFERENTIATION

Inhibition of autophagy, lysosome and VCP function impairs stress granule assembly

(2014) S J Seguin et al.   CELL DEATH AND DIFFERENTIATION

Barcoding heat shock proteins to human diseases: looking beyond the heat shock response

(2014) V. Kakkar et al.   Disease Models & Mechanisms

Synergic prodegradative activity of Bicalutamide and trehalose on the mutant androgen receptor responsible for spinal and bulbar muscular atrophy

(2014) E. Giorgetti et al.   HUMAN MOLECULAR GENETICS

WW Domain of BAG3 Is Required for the Induction of Autophagy in Glioma Cells

(2014) Nana Merabova et al.   JOURNAL OF CELLULAR PHYSIOLOGY

Polyglutamine-expanded androgen receptor interferes with TFEB to elicit autophagy defects in SBMA

(2014) Constanza J Cortes et al.   NATURE NEUROSCIENCE

ALS-related misfolded protein management in motor neurons and muscle cells

(2014) Mariarita Galbiati et al.   NEUROCHEMISTRY INTERNATIONAL

Muscle Expression of Mutant Androgen Receptor Accounts for Systemic and Motor Neuron Disease Phenotypes in Spinal and Bulbar Muscular Atrophy

(2014) Constanza J. Cortes et al.   NEURON

Neuroprotective effect of the chemical chaperone, trehalose in a chronic MPTP-induced Parkinson's disease mouse model

(2014) Sumit Sarkar et al.   NEUROTOXICOLOGY

14-3-3 and aggresome formation: Implications in neurodegenerative diseases

(2014) Baohui Jia et al.   Prion

Peripheral Androgen Receptor Gene Suppression Rescues Disease in Mouse Models of Spinal and Bulbar Muscular Atrophy

(2014) Andrew P. Lieberman et al.   Cell Reports

Androgen-dependent impairment of myogenesis in spinal and bulbar muscular atrophy

(2013) Adriana Malena 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

Oligodendrocyte dysfunction in the pathogenesis of amyotrophic lateral sclerosis

(2013) Thomas Philips et al.   BRAIN

Transcriptional activation of TFEB/ZKSCAN3 target genes underlies enhanced autophagy in spinobulbar muscular atrophy

(2013) J. P. Chua et al.   HUMAN MOLECULAR GENETICS

14-3-3 protein targets misfolded chaperone-associated proteins to aggresomes

(2013) Z. Xu et al.   JOURNAL OF CELL SCIENCE

Trehalose rescues Alzheimer's disease phenotypes in APP/PS1 transgenic mice

(2013) Jing Du et al.   JOURNAL OF PHARMACY AND PHARMACOLOGY

The changing scene of amyotrophic lateral sclerosis

(2013) Wim Robberecht et al.   NATURE REVIEWS NEUROSCIENCE

Clearance of the mutant androgen receptor in motoneuronal models of spinal and bulbar muscular atrophy

(2013) Paola Rusmini et al.   NEUROBIOLOGY OF AGING

DNAJB6 myopathy in an Asian cohort and cytoplasmic/nuclear inclusions

(2013) Takatoshi Sato et al.   NEUROMUSCULAR DISORDERS

Different anti-aggregation and pro-degradative functions of the members of the mammalian sHSP family in neurological disorders

(2013) S. Carra et al.   PHILOSOPHICAL TRANSACTIONS OF THE ROYAL SOCIETY B-BIOLOGICAL SCIENCES

Identification of CHIP as a Novel Causative Gene for Autosomal Recessive Cerebellar Ataxia

(2013) Yuting Shi et al.   PLoS One

Differential autophagy power in the spinal cord and muscle of transgenic ALS mice

(2013) Valeria Crippa et al.   Frontiers in Cellular Neuroscience

Cdc48/p97 promotes degradation of aberrant nascent polypeptides bound to the ribosome

(2013) Rati Verma et al.   eLife

Early onset muscle weakness and disruption of muscle proteins in mouse models of spinal muscular atrophy

(2013) Justin G Boyer et al.   Skeletal Muscle

Stimulation of autophagy is neuroprotective in a mouse model of human tauopathy

(2012) Véronique Schaeffer et al.   Autophagy

The small heat shock proteins αB-crystallin and Hsp27 suppress SOD1 aggregation in vitro

(2012) Justin J. Yerbury et al.   CELL STRESS & CHAPERONES

Genetic Determinants of Amyotrophic Lateral Sclerosis as Therapeutic Targets

(2012) Daryl A. Bosco et al.   CNS & Neurological Disorders-Drug Targets

Trehalose Protects from Aggravation of Amyloid Pathology Induced by Isoflurane Anesthesia in APPswe Mutant Mice

(2012) Juan Perucho et al.   Current Alzheimer Research

NF- B regulates protein quality control after heat stress through modulation of the BAG3-HspB8 complex

(2012) M. Nivon et al.   JOURNAL OF CELL SCIENCE

BAG3 mutations: another cause of giant axonal neuropathy

(2012) Fatima Jaffer et al.   JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM

Mutations affecting the cytoplasmic functions of the co-chaperone DNAJB6 cause limb-girdle muscular dystrophy

(2012) Jaakko Sarparanta et al.   NATURE GENETICS

SQSTM1 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

(2011) Faisal Fecto   ARCHIVES OF NEUROLOGY

BAG3 and friends: Co-chaperones in selective autophagy during aging and disease

(2011) Christian Behl   Autophagy

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

(2011) Xiaojie Zhang et al.   Autophagy

Transduced HSP27 protein protects neuronal cell death by enhancing FALS-associated SOD1 mutant activity

(2011) Jae-Jin An et al.   BMB Reports

BAG3 mediates chaperone-based aggresome-targeting and selective autophagy of misfolded proteins

(2011) Martin Gamerdinger et al.   EMBO REPORTS

Dilated cardiomyopathy-associatedBAG3mutations impair Z-disc assembly and enhance sensitivity to apoptosis in cardiomyocytes

(2011) Takuro Arimura et al.   HUMAN MUTATION

Emerging roles of molecular chaperones and co-chaperones in selective autophagy: focus on BAG proteins

(2011) Martin Gamerdinger et al.   JOURNAL OF MOLECULAR MEDICINE-JMM

Muscle cells and motoneurons differentially remove mutant SOD1 causing familial amyotrophic lateral sclerosis

(2011) Elisa Onesto et al.   JOURNAL OF NEUROCHEMISTRY

Neuroinflammation in amyotrophic lateral sclerosis: role of glial activation in motor neuron disease

(2011) Thomas Philips et al.   LANCET NEUROLOGY

Molecular chaperones in protein folding and proteostasis

(2011) F. Ulrich Hartl et al.   NATURE

The HSPB8-BAG3 chaperone complex is upregulated in astrocytes in the human brain affected by protein aggregation diseases

(2011) K. Seidel et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

BAG3 Directly Interacts with Mutated alphaB-Crystallin to Suppress Its Aggregation and Toxicity

(2011) Akinori Hishiya et al.   PLoS One

Inhibition of α-synuclein aggregation by small heat shock proteins

(2011) Ilona B. Bruinsma et al.   PROTEINS-STRUCTURE FUNCTION AND BIOINFORMATICS

VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD

(2010) Emilie Tresse et al.   Autophagy

Chaperone-Assisted Selective Autophagy Is Essential for Muscle Maintenance

(2010) Verena Arndt et al.   CURRENT BIOLOGY

Mutant HSPB8 causes motor neuron-specific neurite degeneration

(2010) Joy Irobi et al.   HUMAN MOLECULAR GENETICS

The small heat shock protein B8 (HspB8) promotes autophagic removal of misfolded proteins involved in amyotrophic lateral sclerosis (ALS)

(2010) Valeria Crippa et al.   HUMAN MOLECULAR GENETICS

TheLegs at odd angles(Loa) Mutation in Cytoplasmic Dynein Ameliorates Mitochondrial Function in SOD1G93AMouse Model for Motor Neuron Disease

(2010) Ali Morsi El-Kadi et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Identification of theDrosophilaOrtholog of HSPB8

(2010) Serena Carra et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A DNAJB Chaperone Subfamily with HDAC-Dependent Activities Suppresses Toxic Protein Aggregation

(2010) Jurre Hageman et al.   MOLECULAR CELL

Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS

(2010) Andrew C. Elden et al.   NATURE

The HSP70 chaperone machinery: J proteins as drivers of functional specificity

(2010) Harm H. Kampinga et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Proteasomal and autophagic degradative activities in spinal and bulbar muscular atrophy

(2010) Paola Rusmini et al.   NEUROBIOLOGY OF DISEASE

Trehalose ameliorates dopaminergic and tau pathology in parkin deleted/tau overexpressing mice through autophagy activation

(2010) Jose A. Rodríguez-Navarro et al.   NEUROBIOLOGY OF DISEASE

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

Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies

(2009) Manuela Neumann et al.   ACTA NEUROPATHOLOGICA

The stress-inducible HspB8-Bag3 complex induces the eIF2α kinase pathway: Implications for protein quality control and viral factory degradation?

(2009) Serena Carra   Autophagy

Caught in the middle: the role of Bag3 in disease: Figure 1

(2009) Andrea K. McCollum et al.   BIOCHEMICAL JOURNAL

Identification of the key structural motifs involved in HspB8/HspB6–Bag3 interaction

(2009) Margit Fuchs et al.   BIOCHEMICAL JOURNAL

Dismutase-competent SOD1 mutant accumulation in myelinating Schwann cells is not detrimental to normal or transgenic ALS model mice

(2009) B. J. Turner et al.   HUMAN MOLECULAR GENETICS

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

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

Protective Effect of Geranylgeranylacetone via Enhancement of HSPB8 Induction in Desmin-Related Cardiomyopathy

(2009) Atsushi Sanbe et al.   PLoS One

Schwann cells expressing dismutase active mutant SOD1 unexpectedly slow disease progression in ALS mice

(2009) C. S. Lobsiger et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mutation in BAG3 causes severe dominant childhood muscular dystrophy

(2008) Duygu Selcen et al.   ANNALS OF NEUROLOGY

Overexpression of Hsp27 does not influence disease in the mutant SOD1G93Amouse model of amyotrophic lateral sclerosis

(2008) Jyothsna Krishnan et al.   JOURNAL OF NEUROCHEMISTRY

Vesicle associated membrane protein B (VAPB) is decreased in ALS spinal cord

(2008) Georgia Anagnostou et al.   NEUROBIOLOGY OF AGING

Spinal and bulbar muscular atrophy: Skeletal muscle pathology in male patients and heterozygous females

(2007) Gianni Sorarù et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Protective effects of heat shock protein 27 in a model of ALS occur in the early stages of disease progression

(2007) Paul S. Sharp et al.   NEUROBIOLOGY OF DISEASE