Neuromuscular Diseases Due to Chaperone Mutations: A Review and Some New Results

Identification of putative pathogenic single nucleotide variants (SNVs) in genes associated with heart disease in 290 cases of stillbirth

(2019) Ellika Sahlin et al.   PLoS One

Transcription Factor EB Activation Rescues Advanced αB‐Crystallin Mutation‐Induced Cardiomyopathy by Normalizing Desmin Localization

(2019) Xiucui Ma et al.   Journal of the American Heart Association

Local unfolding of the HSP27 monomer regulates chaperone activity

(2019) T. Reid Alderson et al.   Nature Communications

Small heat shock proteins: multifaceted proteins with important implications for life

(2019) Serena Carra et al.   CELL STRESS & CHAPERONES

Neuropathy-causing mutations in HSPB1 impair autophagy by disturbing the formation of SQSTM1/p62 bodies

(2019) Mansour Haidar et al.   Autophagy

Cellular Functions and Mechanisms of Action of Small Heat Shock Proteins

(2019) Axel Mogk et al.   Annual Review of Microbiology

Intrafamilial variability of limb-girdle muscular dystrophy, LGMD1D type

(2019) Judith Zima et al.   European Journal of Medical Genetics

DnaJB6 is a RanGTP-regulated protein required for microtubule organization during mitosis

(2019) M. Rosas-Salvans et al.   JOURNAL OF CELL SCIENCE

Evidence for the impact of BAG3 on electrophysiological activity of primary culture of neonatal cardiomyocytes

(2019) Farzaneh G. Tahrir et al.   JOURNAL OF CELLULAR PHYSIOLOGY

HspB1 phosphorylation regulates its intramolecular dynamics and mechanosensitive molecular chaperone interaction with filamin C

(2019) Miranda P. Collier et al.   Science Advances

Desmin forms toxic, seeding-competent amyloid aggregates that persist in muscle fibers

(2019) Niraja Kedia et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Enhanced autophagic-lysosomal activity and increased BAG3-mediated selective macroautophagy as adaptive response of neuronal cells to chronic oxidative stress

(2019) Debapriya Chakraborty et al.   Redox Biology

A novel homozygous initiation codon variant associated with infantile alpha-Bcrystallinopathy in a Chinese family

(2019) Keze Ma et al.   Molecular Genetics & Genomic Medicine

Haploinsufficiency of mechanistic target of rapamycin ameliorates bag3 cardiomyopathy in adult zebrafish

(2019) Yonghe Ding et al.   Disease Models & Mechanisms

The Molecular Chaperone DNAJB6, but Not DNAJB1, Suppresses the Seeded Aggregation of Alpha-Synuclein in Cells

(2019) Natasja Deshayes et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Age‐related neurodegenerative diseases

(2019) Michael Duggan et al.   JOURNAL OF CELLULAR PHYSIOLOGY

Interplay of disordered and ordered regions of a human small heat shock protein yields an ensemble of 'quasi-ordered' states

(2019) Amanda F Clouser et al.   eLife

Functional and structural characterization of HspB1/Hsp27 from Chinese hamster ovary cells

(2019) Eiryo Sha et al.   FEBS Open Bio

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

(2019) Riccardo Cristofani et al.   Frontiers in Neuroscience

Autophagic and Proteasomal Mediated Removal of Mutant Androgen Receptor in Muscle Models of Spinal and Bulbar Muscular Atrophy

(2019) Maria Elena Cicardi et al.   Frontiers in Endocrinology

Keratin 18 is an integral part of the intermediate filament network in murine skeletal muscle

(2019) Joaquin M. Muriel et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

The Hippo network kinase STK38 contributes to protein homeostasis by inhibiting BAG3-mediated autophagy

(2019) Christina Klimek et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Regulation of small heat shock proteins by hetero-oligomer formation

(2019) Evgeny V. Mymrikov et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Exome sequencing in amyotrophic lateral sclerosis implicates a novel gene, DNAJC7, encoding a heat-shock protein

(2019) Sali M. K. Farhan et al.   NATURE NEUROSCIENCE

Mutations in the J domain of DNAJB6 cause dominant distal myopathy

(2019) Johanna Palmio et al.   NEUROMUSCULAR DISORDERS

Unraveling the structure and dynamics of the human DNAJB6b chaperone by NMR reveals insights into Hsp40-mediated proteostasis

(2019) Theodoros K. Karamanos et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Cellular sequestrases maintain basal Hsp70 capacity ensuring balanced proteostasis

(2019) Chi-ting Ho et al.   Nature Communications

BAG3 regulates multiple myeloma cell proliferation through FOXM1/Rb/E2F axis

(2019) Hua Bai et al.   CANCER GENE THERAPY

Advances in the role and mechanism of BAG3 in dilated cardiomyopathy

(2019) Leiling Liu et al.   HEART FAILURE REVIEWS

BAG3 p.Pro209Ser mutation identified in a Chinese family with Charcot–Marie–Tooth disease

(2019) Jun Fu et al.   JOURNAL OF NEUROLOGY

The 2020 version of the gene table of neuromuscular disorders (nuclear genome)

(2019) Louise Benarroch et al.   NEUROMUSCULAR DISORDERS

SMN complex member Gemin3 self-interacts and has a functional relationship with ALS-linked proteins TDP-43, FUS and Sod1

(2019) Rebecca Cacciottolo et al.   Scientific Reports

Protein–Protein Interactions in the Molecular Chaperone Network

(2018) Rebecca Freilich et al.   ACCOUNTS OF CHEMICAL RESEARCH

The multiple lives of DEAD-box RNA helicase DP103/DDX20/Gemin3

(2018) Frank Curmi et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

Conserved S/T-residues of the human chaperone DNAJB6 are required for effective inhibition of Aβ42 amyloid fibril formation

(2018) Cecilia Månsson et al.   BIOCHEMISTRY

Drosophila Hsp67Bc hot-spot variants alter muscle structure and function

(2018) Jadwiga Jabłońska et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Novel mutations in DNAJB6 cause LGMD1D and distal myopathy in French families

(2018) P. H. Jonson et al.   EUROPEAN JOURNAL OF NEUROLOGY

Decreased ceramide underlies mitochondrial dysfunction in Charcot-Marie-Tooth 2F

(2018) Nicholas U. Schwartz et al.   FASEB JOURNAL

HSPB8 and BAG3 cooperate to promote spatial sequestration of ubiquitinated proteins and coordinate the cellular adaptive response to proteasome insufficiency

(2018) Solenn M. Guilbert et al.   FASEB JOURNAL

Genetic Mutations and Demographic, Clinical, and Morphological Aspects of Myofibrillar Myopathy in a French Cohort

(2018) Alzira Alves de Siqueira Carvalho et al.   Genetic Testing and Molecular Biomarkers

A prion-like domain in Hsp42 drives chaperone-facilitated aggregation of misfolded proteins

(2018) Tomas Grousl et al.   JOURNAL OF CELL BIOLOGY

Haplo-insufficiency of Bcl2-associated athanogene 3 in mice results in progressive left ventricular dysfunction, β-adrenergic insensitivity, and increased apoptosis

(2018) Valerie D. Myers et al.   JOURNAL OF CELLULAR PHYSIOLOGY

TIA1 variant drives myodegeneration in multisystem proteinopathy with SQSTM1 mutations

(2018) YouJin Lee et al.   JOURNAL OF CLINICAL INVESTIGATION

Terminal regions confer plasticity to the tetrameric assembly of human HspB2 and HspB3

(2018) Alice R. Clark et al.   JOURNAL OF MOLECULAR BIOLOGY

Small heat shock protein B3 (HSPB3 ) mutation in an axonal Charcot-Marie-Tooth disease family

(2018) Da E. Nam et al.   JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM

Proteomic profiling of microtubule self-organization in M-phase

(2018) Miquel Rosas-Salvans et al.   MOLECULAR & CELLULAR PROTEOMICS

Dysregulated autophagy in restrictive cardiomyopathy due to Pro209Leu mutation in BAG3

(2018) A. Schänzer et al.   MOLECULAR GENETICS AND METABOLISM

creat229 th ENMC international workshop: Limb girdle muscular dystrophies – nomenclature and reformed classification, 17-19 March 2017, Naarden, The Netherlands

(2018) Volker Straub et al.   NEUROMUSCULAR DISORDERS

BAG3 myopathy is not always associated with cardiomyopathy

(2018) Annarita Ghosh Andersen et al.   NEUROMUSCULAR DISORDERS

Hsp70–Bag3 complex is a hub for proteotoxicity-induced signaling that controls protein aggregation

(2018) Anatoli B. Meriin et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Two Korean Families with Limb-Girdle Muscular Dystrophy Type 1D Associated with DNAJB6 Mutations

(2018) Kitae Kim et al.   YONSEI MEDICAL JOURNAL

Structural modelling of the DNAJB6 oligomeric chaperone shows a peptide-binding cleft lined with conserved S/T-residues at the dimer interface

(2018) Christopher A. G. Söderberg et al.   Scientific Reports

Characterization of human small heat shock protein HSPB1 α-crystallin domain localized mutants associated with hereditary motor neuron diseases

(2018) Stephen D. Weeks et al.   Scientific Reports

CRYAB protects cardiomyocytes against heat stress by preventing caspase-mediated apoptosis and reducing F-actin aggregation

(2018) Bin Yin et al.   CELL STRESS & CHAPERONES

Heat Shock Protein B8 (HSPB8) Reduces Oxygen-Glucose Deprivation/Reperfusion Injury via the Induction of Mitophagy

(2018) Fazhao Li et al.   CELLULAR PHYSIOLOGY AND BIOCHEMISTRY

BAG3 mutation in a patient with atypical phenotypes of myofibrillar myopathy and Charcot–Marie–Tooth disease

(2018) Seung Ju Kim et al.   Genes & Genomics

Mechanistic insights into the switch of αB-crystallin chaperone activity and self-multimerization

(2018) Zhenying Liu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

GSK3-β promotes calpain-1–mediated desmin filament depolymerization and myofibril loss in atrophy

(2018) Dina Aweida et al.   JOURNAL OF CELL BIOLOGY

Human DnaJB6 anti-amyloid chaperone protects yeast from polyglutamine toxicity separately from spatial segregation of aggregates

(2018) Jyotsna Kumar et al.   MOLECULAR AND CELLULAR BIOLOGY

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

(2018) Maria Elena Cicardi et al.   Scientific Reports

P209L Mutation in BAG3 Does Not Cause Cardiomyopathy in Mice

(2018) Xi Fang et al.   AMERICAN JOURNAL OF PHYSIOLOGY-HEART AND CIRCULATORY PHYSIOLOGY

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

(2018) Paola Rusmini et al.   Autophagy

Function, evolution, and structure of J-domain proteins

(2018) Harm H. Kampinga et al.   CELL STRESS & CHAPERONES

It takes a dimer to tango: Oligomeric small heat shock proteins dissociate to capture substrate

(2018) Indu Santhanagopalan et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Small heat shock proteins: Simplicity meets complexity

(2018) Martin Haslbeck et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

HSPB5 engages multiple states of a destabilized client to enhance chaperone activity in a stress-dependent manner.

(2018) Scott P. Delbecq et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Single-copy expression of an amyotrophic lateral sclerosis-linked TDP-43 mutation (M337V) in BAC transgenic mice leads to altered stress granule dynamics and progressive motor dysfunction

(2018) David Gordon et al.   NEUROBIOLOGY OF DISEASE

Competing protein-protein interactions regulate binding of Hsp27 to its client protein tau

(2018) Rebecca Freilich et al.   Nature Communications

Myopathy associated BAG3 mutations lead to protein aggregation by stalling Hsp70 networks

(2018) Melanie Meister-Broekema et al.   Nature Communications

Genetic landscape and novel disease mechanisms from a large LGMD cohort of 4656 patients

(2018) Babi Ramesh Reddy Nallamilli et al.   Annals of Clinical and Translational Neurology

Astrocytic expression of the chaperone DNAJB6 results in non-cell autonomous protection in Huntington’s disease

(2018) Matteo Bason et al.   NEUROBIOLOGY OF DISEASE

Altered TDP-43-dependent splicing in HSPB8 -related distal hereditary motor neuropathy and myofibrillar myopathy

(2017) A. Cortese et al.   EUROPEAN JOURNAL OF NEUROLOGY

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

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

Mitochondrial deficits and abnormal mitochondrial retrograde axonal transport play a role in the pathogenesis of mutant Hsp27-induced Charcot Marie Tooth Disease

(2017) Bernadett Kalmar et al.   HUMAN MOLECULAR GENETICS

Axonal Neuropathies due to Mutations in Small Heat Shock Proteins: Clinical, Genetic, and Functional Insights into Novel Mutations

(2017) Andoni Echaniz-Laguna et al.   HUMAN MUTATION

The novel αB-crystallin (CRYAB ) mutation p.D109G causes restrictive cardiomyopathy

(2017) Andreas Brodehl et al.   HUMAN MUTATION

Characteristic Posterior-dominant Lower Limb Muscle Involvement in Limb-girdle Muscular Dystrophy due to a DNAJB6 Phe93Leu Mutation

(2017) Yuta Kojima et al.   INTERNAL MEDICINE

Modulation of mutant Huntingtin aggregates and toxicity by human myeloid leukemia factors

(2017) Manisha Banerjee et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Loss-of-function mutations in co-chaperone BAG3 destabilize small HSPs and cause cardiomyopathy

(2017) Xi Fang et al.   JOURNAL OF CLINICAL INVESTIGATION

TFEB activation protects against cardiac proteotoxicity via increasing autophagic flux

(2017) Bo Pan et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

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

Myeloid Leukemia Factor Acts in a Chaperone Complex to Regulate Transcription Factor Stability and Gene Expression

(2017) Jamie O. Dyer et al.   JOURNAL OF MOLECULAR BIOLOGY

Mutations in BAG3 cause adult-onset Charcot-Marie-Tooth disease

(2017) Michael Shy et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Mechanical signals activate p38 MAPK pathway-dependent reinforcement of actin via mechanosensitive HspB1

(2017) Laura Hoffman et al.   MOLECULAR BIOLOGY OF THE CELL

Rigid spine syndrome associated with sensory-motor axonal neuropathy resembling Charcot-Marie-Tooth disease is characteristic of Bcl-2-associated athanogene-3 gene mutations even without cardiac involvement

(2017) Jean-Baptiste Noury et al.   MUSCLE & NERVE

Architecture of the human interactome defines protein communities and disease networks

(2017) Edward L. Huttlin et al.   NATURE

Site-specific mapping of the human SUMO proteome reveals co-modification with phosphorylation

(2017) Ivo A Hendriks et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Pilot phenotype and natural history study of hereditary neuropathies caused by mutations in the HSPB1 gene

(2017) Alexander M. Rossor et al.   NEUROMUSCULAR DISORDERS

Common and Distinctive Functions of the Hippo Effectors Taz and Yap in Skeletal Muscle Stem Cell Function

(2017) Congshan Sun et al.   STEM CELLS

How Do J-Proteins Get Hsp70 to Do So Many Different Things?

(2017) Elizabeth A. Craig et al.   TRENDS IN BIOCHEMICAL SCIENCES

HSPB1 facilitates ERK-mediated phosphorylation and degradation of BIM to attenuate endoplasmic reticulum stress-induced apoptosis

(2017) Donna Kennedy et al.   Cell Death & Disease

Site-specific mapping of the human SUMO proteome reveals co-modification with phosphorylation

(2017) Ivo A Hendriks et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Aberrant Compartment Formation by HSPB2 Mislocalizes Lamin A and Compromises Nuclear Integrity and Function

(2017) Federica F. Morelli et al.   Cell Reports

Tat-HSP22 inhibits oxidative stress-induced hippocampal neuronal cell death by regulation of the mitochondrial pathway

(2017) Hyo Sang Jo et al.   Molecular Brain

Control of RUNX-induced repression of Notch signaling by MLF and its partner DnaJ-1 during Drosophila hematopoiesis

(2017) Marion Miller et al.   PLoS Genetics

Evolution of an intricate J-protein network driving protein disaggregation in eukaryotes

(2017) Nadinath B Nillegoda et al.   eLife

The Role of the Multifunctional BAG3 Protein in Cellular Protein Quality Control and in Disease

(2017) Elisabeth Stürner et al.   Frontiers in Molecular Neuroscience

L-3-n-Butylphthalide Protects HSPB8 K141N Mutation-Induced Oxidative Stress by Modulating the Mitochondrial Apoptotic and Nrf2 Pathways

(2017) Xiao-Dong Yang et al.   Frontiers in Neuroscience

Mutant HSPB1 causes loss of translational repression by binding to PCBP1, an RNA binding protein with a possible role in neurodegenerative disease

(2017) Thomas Geuens et al.   Acta Neuropathologica Communications

The molecular chaperones DNAJB6 and Hsp70 cooperate to suppress α-synuclein aggregation

(2017) Francesco A. Aprile et al.   Scientific Reports

Mutant desmin substantially perturbs mitochondrial morphology, function and maintenance in skeletal muscle tissue

(2016) Lilli Winter et al.   ACTA NEUROPATHOLOGICA

The role ofDNAJB2in amyotrophic lateral sclerosis

(2016) Marina Frasquet et al.   BRAIN

The heat shock response plays an important role in TDP-43 clearance: evidence for dysfunction in amyotrophic lateral sclerosis

(2016) Han-Jou Chen et al.   BRAIN

Interaction of small heat shock proteins with light component of neurofilaments (NFL)

(2016) Victoria V. Nefedova et al.   CELL STRESS & CHAPERONES

HSPB3 protein is expressed in motoneurons and induces their survival after lesion-induced degeneration

(2016) Veronica La Padula et al.   EXPERIMENTAL NEUROLOGY

Protein kinase CK2 modulates HSJ1 function through phosphorylation of the UIM2 domain

(2016) Daniele Ottaviani et al.   HUMAN MOLECULAR GENETICS

Genetic interaction of hnRNPA2B1 and DNAJB6 in aDrosophilamodel of multisystem proteinopathy

(2016) Songqing Li et al.   HUMAN MOLECULAR GENETICS

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

Identification of a LargeDNAJB2Deletion in a Family with Spinal Muscular Atrophy and Parkinsonism

(2016) Elena Sanchez et al.   HUMAN MUTATION

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

(2016) Simona Capponi et al.   HUMAN MUTATION

The Human 343delT HSPB5 Chaperone Associated with Early-onset Skeletal Myopathy Causes Defects in Protein Solubility

(2016) Katie A. Mitzelfelt et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Non-canonical Interactions between Heat Shock Cognate Protein 70 (Hsc70) and Bcl2-associated Anthanogene (BAG) Co-Chaperones Are Important for Client Release

(2016) Jennifer N. Rauch et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Chaperone Activity and Substrate Spectrum of Human Small Heat Shock Proteins

(2016) Evgeny V. Mymrikov et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Desmin and αB-crystallin interplay in the maintenance of mitochondrial homeostasis and cardiomyocyte survival

(2016) Antigoni Diokmetzidou et al.   JOURNAL OF CELL SCIENCE

Evidence for the Role of BAG3 in Mitochondrial Quality Control in Cardiomyocytes

(2016) Farzaneh G. Tahrir et al.   JOURNAL OF CELLULAR PHYSIOLOGY

BAG3 regulates contractility and Ca2+ homeostasis in adult mouse ventricular myocytes

(2016) Arthur M. Feldman et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

Assessment of Targeted Next-Generation Sequencing as a Tool for the Diagnosis of Charcot-Marie-Tooth Disease and Hereditary Motor Neuropathy

(2016) Vincenzo Lupo et al.   JOURNAL OF MOLECULAR DIAGNOSTICS

Exome sequencing identifies variants in two genes encoding the LIM-proteins NRAP and FHL1 in an Italian patient with BAG3 myofibrillar myopathy

(2016) Francesca D’Avila et al.   JOURNAL OF MUSCLE RESEARCH AND CELL MOTILITY

Phenotype and natural history of inherited neuropathies caused byHSJ1c.352+1G>A mutation

(2016) M Frasquet et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Cardioprotection of exercise preconditioning involving heat shock protein 70 and concurrent autophagy: a potential chaperone-assisted selective macroautophagy effect

(2016) Yang Yuan et al.   Journal of Physiological Sciences

NFκB is a central regulator of protein quality control in response to protein aggregation stresses via autophagy modulation

(2016) Mathieu Nivon et al.   MOLECULAR BIOLOGY OF THE CELL

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

(2016) Massimo Ganassi et al.   MOLECULAR CELL

The S/T-Rich Motif in the DNAJB6 Chaperone Delays Polyglutamine Aggregation and the Onset of Disease in a Mouse Model

(2016) Vaishali Kakkar et al.   MOLECULAR CELL

Target-enrichment sequencing and copy number evaluation in inherited polyneuropathy

(2016) Wei Wang et al.   NEUROLOGY

Distal hereditary motor neuropathy with HSJ1 chaperone mutation, presenting with peripheral motor neuropathy, associated to parkinsonism, and cerebellar ataxia. Case report

(2016) Helio Teive et al.   PARKINSONISM & RELATED DISORDERS

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

(2016) Christian Behl   TRENDS IN PHARMACOLOGICAL SCIENCES

Small heat shock proteins sequester misfolding proteins in near-native conformation for cellular protection and efficient refolding

(2016) Sophia Ungelenk et al.   Nature Communications

Emerging roles and underlying molecular mechanisms of DNAJB6 in cancer

(2016) Erhong Meng et al.   Oncotarget

HDAC6 Inhibitors Rescued the Defective Axonal Mitochondrial Movement in Motor Neurons Derived from the Induced Pluripotent Stem Cells of Peripheral Neuropathy Patients with HSPB1 Mutation

(2016) Ji-Yon Kim et al.   Stem Cells International

The beneficial role of proteolysis in skeletal muscle growth and stress adaptation

(2016) Ryan A. V. Bell et al.   Skeletal Muscle

Diagnostically important muscle pathology in DNAJB6 mutated LGMD1D

(2016) Satu Sandell et al.   Acta Neuropathologica Communications

Molecular Mechanisms of Amyloid Oligomers Toxicity

(2016) Rakez Kayed et al.   JOURNAL OF ALZHEIMERS DISEASE

Familial Dilated Cardiomyopathy Caused by a Novel Frameshift in the BAG3 Gene

(2016) Rocio Toro et al.   PLoS One

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

Versatile members of the DNAJ family show Hsp70 dependent anti-aggregation activity on RING1 mutant parkin C289G

(2016) Vaishali Kakkar et al.   Scientific Reports

Bicyclic-Capped Histone Deacetylase 6 Inhibitors with Improved Activity in a Model of Axonal Charcot–Marie–Tooth Disease

(2015) Sida Shen et al.   ACS Chemical Neuroscience

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

(2015) Anna Ulbricht et al.   Autophagy

BAG3 affects the nucleocytoplasmic shuttling of HSF1 upon heat stress

(2015) Young-Hee Jin et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

A Mechanism of Subunit Recruitment in Human Small Heat Shock Protein Oligomers

(2015) Scott P. Delbecq et al.   BIOCHEMISTRY

Widespread Proteome Remodeling and Aggregation in Aging C. elegans

(2015) Dirk M. Walther et al.   CELL

Phosphomimics Destabilize Hsp27 Oligomeric Assemblies and Enhance Chaperone Activity

(2015) Blagojce Jovcevski et al.   CHEMISTRY & BIOLOGY

HspB8 is Neuroprotective during Oxygen Glucose Deprivation and Reperfusion

(2015) Binbin Yang et al.   CURRENT NEUROVASCULAR RESEARCH

Drosophila small heat shock protein CryAB ensures structural integrity of developing muscles, and proper muscle and heart performance

(2015) I. Wojtowicz et al.   DEVELOPMENT

Myofibrillar disruption and RNA-binding protein aggregation in a mouse model of limb-girdle muscular dystrophy 1D

(2015) Rocio Bengoechea et al.   HUMAN MOLECULAR GENETICS

Myofibrillar instability exacerbated by acute exercise in filaminopathy

(2015) Frédéric Chevessier et al.   HUMAN MOLECULAR GENETICS

Human J-protein DnaJB6b Cures a Subset ofSaccharomyces cerevisiaePrions and Selectively Blocks Assembly of Structurally Related Amyloids

(2015) Michael Reidy et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Structure of the α-crystallin domain from the redox-sensitive chaperone, HSPB1

(2015) Ponni Rajagopal et al.   JOURNAL OF BIOMOLECULAR NMR

BAG3-related myopathy, polyneuropathy and cardiomyopathy with long QT syndrome

(2015) Anna Kostera-Pruszczyk et al.   JOURNAL OF MUSCLE RESEARCH AND CELL MOTILITY

A novel heat shock protein 27 homozygous mutation: widening of the continuum between MND/dHMN/CMT2

(2015) Marina Scarlato et al.   JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM

Crucial HSP70 co-chaperone complex unlocks metazoan protein disaggregation

(2015) Nadinath B. Nillegoda et al.   NATURE

The chaperone αB-crystallin uses different interfaces to capture an amorphous and an amyloid client

(2015) Andi Mainz et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Mutations inHSPB8causing a new phenotype of distal myopathy and motor neuropathy

(2015) Roula Ghaoui et al.   NEUROLOGY

Novel mutations in DNAJB6 gene cause a very severe early-onset limb-girdle muscular dystrophy 1D disease

(2015) Johanna Palmio et al.   NEUROMUSCULAR DISORDERS

A novel mutation in DNAJB6, p.(Phe91Leu), in childhood-onset LGMD1D with a severe phenotype

(2015) Tai-Seung Nam et al.   NEUROMUSCULAR DISORDERS

BAG3 myofibrillar myopathy presenting with cardiomyopathy

(2015) Chamindra G. Konersman et al.   NEUROMUSCULAR DISORDERS

The chaperone αB-crystallin uses different interfaces to capture an amorphous and an amyloid client

(2015) Andi Mainz et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

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

Exome Sequence Analysis Suggests that Genetic Burden Contributes to Phenotypic Variability and Complex Neuropathy

(2015) Claudia Gonzaga-Jauregui et al.   Cell Reports

A conserved histidine modulates HSPB5 structure to trigger chaperone activity in response to stress-related acidosis

(2015) Ponni Rajagopal et al.   eLife

Complete loss of the DNAJB6 G/F domain and novel missense mutations cause distal-onset DNAJB6 myopathy

(2015) Alessandra Ruggieri et al.   Acta Neuropathologica Communications

Heat Shock Protein 22 (Hsp22) Regulates Oxidative Phosphorylation upon Its Mitochondrial Translocation with the Inducible Nitric Oxide Synthase in Mammalian Heart

(2015) Eman Rashed et al.   PLoS One

Characterization of Mutants of Human Small Heat Shock Protein HspB1 Carrying Replacements in the N-Terminal Domain and Associated with Hereditary Motor Neuron Diseases

(2015) Lydia K. Muranova et al.   PLoS One

Characterization of the Cardiac Overexpression of HSPB2 Reveals Mitochondrial and Myogenic Roles Supported by a Cardiac HspB2 Interactome

(2015) Julianne H. Grose et al.   PLoS One

The small heat shock protein B8 (HSPB8) confers resistance to bortezomib by promoting autophagic removal of misfolded proteins in multiple myeloma cells

(2015) Mohamed-Amine Hamouda et al.   Oncotarget

Zebrafish models of BAG3 myofibrillar myopathy suggest a toxic gain of function leading to BAG3 insufficiency

(2014) Avnika A. Ruparelia et al.   ACTA NEUROPATHOLOGICA

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

Characterization of human small heat shock protein HspB1 that carries C-terminal domain mutations associated with hereditary motor neuron diseases

(2014) Anna S. Chalova et al.   BIOCHIMICA ET BIOPHYSICA ACTA-PROTEINS AND PROTEOMICS

A Quantitative Chaperone Interaction Network Reveals the Architecture of Cellular Protein Homeostasis Pathways

(2014) Mikko Taipale et al.   CELL

Desmin in muscle and associated diseases: beyond the structural function

(2014) Karim Hnia et al.   CELL AND TISSUE RESEARCH

Effect of disulfide crosslinking on thermal transitions and chaperone-like activity of human small heat shock protein HspB1

(2014) Anna S. Chalova et al.   CELL STRESS & CHAPERONES

Pathology of frontotemporal dementia with limb girdle muscular dystrophy caused by a DNAJB6 mutation

(2014) Ichiro Yabe et al.   CLINICAL NEUROLOGY AND NEUROSURGERY

Contribution of small heat shock proteins to muscle development and function

(2014) Magda Dubińska-Magiera et al.   FEBS LETTERS

Myopathy-causing Mutations in an HSP40 Chaperone Disrupt Processing of Specific Client Conformers

(2014) Kevin C. Stein et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Interaction of the Molecular Chaperone DNAJB6 with Growing Amyloid-beta 42 (Aβ42) Aggregates Leads to Sub-stoichiometric Inhibition of Amyloid Formation

(2014) Cecilia Månsson et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Crystallin-αB Regulates Skeletal Muscle Homeostasis via Modulation of Argonaute2 Activity

(2014) Ronald L. Neppl et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Human myocytes are protected from titin aggregation-induced stiffening by small heat shock proteins

(2014) Sebastian Kötter et al.   JOURNAL OF CELL BIOLOGY

The BAG3 gene variants in Polish patients with dilated cardiomyopathy: four novel mutations and a genotype-phenotype correlation

(2014) Maria Franaszczyk et al.   Journal of Translational Medicine

The Functional Landscape of Hsp27 Reveals New Cellular Processes such as DNA Repair and Alternative Splicing and Proposes Novel Anticancer Targets

(2014) Maria Katsogiannou et al.   MOLECULAR & CELLULAR PROTEOMICS

A novel transgenic mouse model of Chinese Charcot-Marie-Tooth disease type 2L

(2014) Ruxu Zhang et al.   Neural Regeneration Research

HSJ1-related hereditary neuropathies: Novel mutations and extended clinical spectrum

(2014) B. Gess et al.   NEUROLOGY

Exome sequencing identifies a DNAJB6 mutation in a family with dominantly-inherited limb-girdle muscular dystrophy

(2014) Julien Couthouis et al.   NEUROMUSCULAR DISORDERS

Exome-wide Rare Variant Analysis Identifies TUBA4A Mutations Associated with Familial ALS

(2014) Bradley N. Smith et al.   NEURON

Unusual multisystemic involvement and a novel BAG3 mutation revealed by NGS screening in a large cohort of myofibrillar myopathies

(2014) Anna-Lena Semmler et al.   Orphanet Journal of Rare Diseases

Mapping of SUMO sites and analysis of SUMOylation changes induced by external stimuli

(2014) F. Impens et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Bag6 complex contains a minimal tail-anchor–targeting module and a mock BAG domain

(2014) Jee-Young Mock et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Cell biological roles of αB-crystallin

(2014) Wilbert C. Boelens   PROGRESS IN BIOPHYSICS & MOLECULAR BIOLOGY

The allelic spectrum of Charcot-Marie-Tooth disease in over 17,000 individuals with neuropathy

(2014) Christina DiVincenzo et al.   Molecular Genetics & Genomic Medicine

Charcot–Marie–Tooth causing HSPB1 mutations increase Cdk5-mediated phosphorylation of neurofilaments

(2013) Anne Holmgren et al.   ACTA NEUROPATHOLOGICA

Aggregate-Prone R120GCRYAB Triggers Multifaceted Modifications of the Thioredoxin System

(2013) Soumyajit Banerjee Mustafi et al.   ANTIOXIDANTS & REDOX SIGNALING

Structure and properties of G84R and L99M mutants of human small heat shock protein HspB1 correlating with motor neuropathy

(2013) Victoria V. Nefedova et al.   ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS

Physico-chemical properties of R140G and K141Q mutants of human small heat shock protein HspB1 associated with hereditary peripheral neuropathies

(2013) Victoria V. Nefedova et al.   BIOCHIMIE

The Small Heat Shock Protein Hsp27 Affects Assembly Dynamics and Structure of Keratin Intermediate Filament Networks

(2013) Jona Kayser et al.   BIOPHYSICAL JOURNAL

DNAJB6 is a peptide-binding chaperone which can suppress amyloid fibrillation of polyglutamine peptides at substoichiometric molar ratios

(2013) Cecilia Månsson et al.   CELL STRESS & CHAPERONES

Congenital posterior pole cataract and adult onset dilating cardiomyopathy: expanding the phenotype of αB-crystallinopathies

(2013) J.J. van der Smagt et al.   CLINICAL GENETICS

Cellular Mechanotransduction Relies on Tension-Induced and Chaperone-Assisted Autophagy

(2013) Anna Ulbricht et al.   CURRENT BIOLOGY

One size does not fit all: The oligomeric states of αB crystallin

(2013) Scott P. Delbecq et al.   FEBS LETTERS

Prion-like nuclear aggregation of TDP-43 during heat shock is regulated by HSP40/70 chaperones

(2013) Maria Udan-Johns et al.   HUMAN MOLECULAR GENETICS

Structural and Functional Aspects of Hetero-oligomers Formed by the Small Heat Shock Proteins αB-Crystallin and HSP27

(2013) J. Andrew Aquilina et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Phenotype of Cardiomyopathy in Cardiac-specific Heat Shock Protein B8 K141N Transgenic Mouse

(2013) Atsushi Sanbe et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The DNAJB6 and DNAJB8 Protein Chaperones Prevent Intracellular Aggregation of Polyglutamine Peptides

(2013) Judith Gillis et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Binding of Human Nucleotide Exchange Factors to Heat Shock Protein 70 (Hsp70) Generates Functionally Distinct Complexesin Vitro

(2013) Jennifer N. Rauch et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

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

Enhanced autophagy ameliorates cardiac proteinopathy

(2013) Md. Shenuarin Bhuiyan et al.   JOURNAL OF CLINICAL INVESTIGATION

Bcl2-associated Athanogene 3 Interactome Analysis Reveals a New Role in Modulating Proteasome Activity

(2013) Ying Chen et al.   MOLECULAR & CELLULAR PROTEOMICS

DNAJB6 myopathy: A vacuolar myopathy with childhood onset

(2013) Gerson Suarez-Cedeno et al.   MUSCLE & NERVE

Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS

(2013) Hong Joo Kim et al.   NATURE

Spatial sequestration of misfolded proteins by a dynamic chaperone pathway enhances cellular fitness during stress

(2013) Stéphanie Escusa-Toret et al.   NATURE CELL BIOLOGY

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

A novel Lys141Thr mutation in small heat shock protein 22 (HSPB8) gene in Charcot–Marie–Tooth disease type 2L

(2013) Khriezhanuo Nakhro et al.   NEUROMUSCULAR DISORDERS

The specificity of the interaction between  B-crystallin and desmin filaments and its impact on filament aggregation and cell viability

(2013) J. L. Elliott et al.   PHILOSOPHICAL TRANSACTIONS OF THE ROYAL SOCIETY B-BIOLOGICAL SCIENCES

HSPB1 Facilitates the Formation of Non-Centrosomal Microtubules

(2013) Leonardo Almeida-Souza et al.   PLoS One

Pseudophosphorylated αB-Crystallin Is a Nuclear Chaperone Imported into the Nucleus with Help of the SMN Complex

(2013) John den Engelsman et al.   PLoS One

Analysis of the Dominant Effects Mediated by Wild Type or R120G Mutant of αB-crystallin (HspB5) towards Hsp27 (HspB1)

(2013) Stéphanie Simon et al.   PLoS One

Molecular Chaperone Mediated Late-Stage Neuroprotection in the SOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis

(2013) Sergey S. Novoselov et al.   PLoS One

Cell Stress Promotes the Association of Phosphorylated HspB1 with F-Actin

(2013) Joseph P. Clarke et al.   PLoS One

Regulated structural transitions unleash the chaperone activity of  B-crystallin

(2013) J. Peschek et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Welander distal myopathy is caused by a mutation in the RNA-binding protein TIA1

(2012) Peter Hackman et al.   ANNALS OF NEUROLOGY

Sequence, Structure, and Dynamic Determinants of Hsp27 (HspB1) Equilibrium Dissociation Are Encoded by the N-Terminal Domain

(2012) Ezelle T. McDonald et al.   BIOCHEMISTRY

Utilization of fluorescent chimeras for investigation of heterooligomeric complexes formed by human small heat shock proteins

(2012) Petr N. Datskevich et al.   BIOCHIMIE

Suppression of protein aggregation by chaperone modification of high molecular weight complexes

(2012) John Labbadia et al.   BRAIN

Structural Aspects and Chaperone Activity of Human HspB3: Role of the “C-Terminal Extension”

(2012) Abhishek Asthana et al.   CELL BIOCHEMISTRY AND BIOPHYSICS

The Role of Intrinsically Disordered Regions in the Structure and Functioning of Small Heat Shock Proteins

(2012) Maria V. Sudnitsyna et al.   CURRENT PROTEIN & PEPTIDE SCIENCE

Binding determinants of the small heat shock protein, αB-crystallin: recognition of the ‘IxI’ motif

(2012) Scott P Delbecq et al.   EMBO JOURNAL

Cellular stress stimulates nuclear localization signal (NLS) independent nuclear transport of MRJ

(2012) Joel F. Andrews et al.   EXPERIMENTAL CELL RESEARCH

Cardiac H11 kinase/Hsp22 stimulates oxidative phosphorylation and modulates mitochondrial reactive oxygen species production: Involvement of a nitric oxide-dependent mechanism

(2012) Lydie Laure et al.   FREE RADICAL BIOLOGY AND MEDICINE

Small heat shock proteins HSP27 (HspB1), αB-crystallin (HspB5) and HSP22 (HspB8) as regulators of cell death

(2012) Julie Acunzo et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

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

Mutant HSPB1 overexpression in neurons is sufficient to cause age-related motor neuronopathy in mice

(2012) Amit K. Srivastava et al.   NEUROBIOLOGY OF DISEASE

Mutant HSPB8 causes protein aggregates and a reduced mitochondrial membrane potential in dermal fibroblasts from distal hereditary motor neuropathy patients

(2012) Joy Irobi et al.   NEUROMUSCULAR DISORDERS

DNAJB6 chaperones PP2A mediated dephosphorylation of GSK3β to downregulate β-catenin transcription target, osteopontin

(2012) A Mitra et al.   ONCOGENE

Chaperones in autophagy

(2012) Susmita Kaushik et al.   PHARMACOLOGICAL RESEARCH

HspB2/Myotonic Dystrophy Protein Kinase Binding Protein (MKBP) as a Novel Molecular Chaperone: Structural and Functional Aspects

(2012) Sankaralingam Prabhu et al.   PLoS One

HSPB2 Is Dispensable for the Cardiac Hypertrophic Response but Reduces Mitochondrial Energetics following Pressure Overload In Mice

(2012) Takahiro Ishiwata et al.   PLoS One

Genome-wide Studies of Copy Number Variation and Exome Sequencing Identify Rare Variants in BAG3 as a Cause of Dilated Cardiomyopathy

(2011) Nadine Norton et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Absolute amounts and diffusibility of HSP72, HSP25, and αB-crystallin in fast- and slow-twitch skeletal muscle fibers of rat

(2011) Noni T. Larkins et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Preemptive conditioning of the swine heart by H11 kinase/Hsp22 provides cardiac protection through inducible nitric oxide synthase

(2011) Li Chen et al.   AMERICAN JOURNAL OF PHYSIOLOGY-HEART AND CIRCULATORY PHYSIOLOGY

A rare recessive distal hereditary motor neuropathy with HSJ1 chaperone mutation

(2011) Sergiu C. Blumen et al.   ANNALS OF NEUROLOGY

Exome sequencing reveals DNAJB6 mutations in dominantly-inherited myopathy

(2011) Matthew B. Harms et al.   ANNALS OF NEUROLOGY

Biochemical characterization of small heat shock protein HspB8 (Hsp22)–Bag3 interaction

(2011) Anton A. Shemetov et al.   ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS

The diverse members of the mammalian HSP70 machine show distinct chaperone-like activities

(2011) Jurre Hageman et al.   BIOCHEMICAL JOURNAL

Phosphorylation by cyclic AMP-dependent protein kinase inhibits chaperone-like activity of human HSP22 in vitro

(2011) A. A. Shemetov et al.   BIOCHEMISTRY-MOSCOW

Heterooligomeric complexes of human small heat shock proteins

(2011) Evgeny V. Mymrikov et al.   CELL STRESS & CHAPERONES

H11 Kinase/Heat Shock Protein 22 Deletion Impairs Both Nuclear and Mitochondrial Functions of STAT3 and Accelerates the Transition Into Heart Failure on Cardiac Overload

(2011) Hongyu Qiu et al.   CIRCULATION

Atg7 Induces Basal Autophagy and Rescues Autophagic Deficiency in CryAB R120G Cardiomyocytes

(2011) J. Scott Pattison et al.   CIRCULATION RESEARCH

BAG3-related myofibrillar myopathy in a Chinese family

(2011) HC Lee et al.   CLINICAL GENETICS

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

(2011) Martin Gamerdinger et al.   EMBO REPORTS

A genome-wide association study identifies two loci associated with heart failure due to dilated cardiomyopathy

(2011) Eric Villard et al.   EUROPEAN HEART JOURNAL

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

(2011) Takuro Arimura et al.   HUMAN MUTATION

Structural and Mechanistic Implications of Metal Binding in the Small Heat-shock Protein αB-crystallin

(2011) Andi Mainz et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Hsp42 is required for sequestration of protein aggregates into deposition sites inSaccharomyces cerevisiae

(2011) Sebastian Specht et al.   JOURNAL OF CELL BIOLOGY

Enhancement of proteasomal function protects against cardiac proteinopathy and ischemia/reperfusion injury in mice

(2011) Jie Li et al.   JOURNAL OF CLINICAL INVESTIGATION

The Neurodegenerative-Disease-Related Protein Sacsin Is a Molecular Chaperone

(2011) John F. Anderson et al.   JOURNAL OF MOLECULAR BIOLOGY

Crystal Structure of R120G Disease Mutant of Human αB-Crystallin Domain Dimer Shows Closure of a Groove

(2011) A.R. Clark et al.   JOURNAL OF MOLECULAR BIOLOGY

HspB8 mutation causing hereditary distal motor neuropathy impairs lysosomal delivery of autophagosomes

(2011) Alice S. Kwok et al.   JOURNAL OF NEUROCHEMISTRY

Small Heat-Shock Protein HSPB1 Mutants Stabilize Microtubules in Charcot-Marie-Tooth Neuropathy

(2011) L. Almeida-Souza et al.   JOURNAL OF NEUROSCIENCE

HSPB1 and HSPB8 in inherited neuropathies: study of an Italian cohort of dHMN and CMT2 patients

(2011) Simona Capponi et al.   JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM

Phosphorylation of human small heat shock protein HspB8 (Hsp22) by ERK1 protein kinase

(2011) Anton A. Shemetov et al.   MOLECULAR AND CELLULAR BIOCHEMISTRY

The Cytosolic Chaperone Hsc70 Promotes Traffic to the Cell Surface of Intracellular Retained Melanocortin-4 Receptor Mutants

(2011) Eirini Meimaridou et al.   MOLECULAR ENDOCRINOLOGY

HDAC6 inhibitors reverse axonal loss in a mouse model of mutant HSPB1–induced Charcot-Marie-Tooth disease

(2011) Constantin d'Ydewalle et al.   NATURE MEDICINE

A novel CRYAB mutation resulting in multisystemic disease

(2011) Sabrina Sacconi et al.   NEUROMUSCULAR DISORDERS

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

A Knock-In Mouse Model for the R120G Mutation of αB-Crystallin Recapitulates Human Hereditary Myopathy and Cataracts

(2011) Usha P. Andley et al.   PLoS One

Co-Chaperone HSJ1a Dually Regulates the Proteasomal Degradation of Ataxin-3

(2011) Xue-Chao Gao et al.   PLoS One

The Mutational Spectrum in a Cohort of Charcot-Marie-Tooth Disease Type 2 among the Han Chinese in Taiwan

(2011) Kon-Ping Lin et al.   PLoS One

N-terminal domain of  B-crystallin provides a conformational switch for multimerization and structural heterogeneity

(2011) S. Jehle et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Inhibition of α-synuclein aggregation by small heat shock proteins

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

BAG3: a multifaceted protein that regulates major cell pathways

(2011) A Rosati et al.   Cell Death & Disease

DNAJB2 Expression in Normal and Diseased Human and Mouse Skeletal Muscle

(2010) Kristl G. Claeys et al.   AMERICAN JOURNAL OF PATHOLOGY

Infantile muscular dystrophy in Canadian aboriginals is an αB-crystallinopathy

(2010) Marc R. Del Bigio et al.   ANNALS OF NEUROLOGY

Sustained Activation of Nuclear Erythroid 2-Related Factor 2/Antioxidant Response Element Signaling Promotes Reductive Stress in the Human Mutant Protein Aggregation Cardiomyopathy in Mice

(2010) Namakkal Soorappan Rajasekaran et al.   ANTIOXIDANTS & REDOX SIGNALING

Abnormal interaction of motor neuropathy-associated mutant HspB8 (Hsp22) forms with the RNA helicase Ddx20 (gemin3)

(2010) Xiankui Sun et al.   CELL STRESS & CHAPERONES

BAG3 and Hsc70 Interact With Actin Capping Protein CapZ to Maintain Myofibrillar Integrity Under Mechanical Stress

(2010) Akinori Hishiya et al.   CIRCULATION RESEARCH

Manipulation of Death Pathways in Desmin-Related Cardiomyopathy

(2010) Alina Maloyan et al.   CIRCULATION RESEARCH

Chaperone-Assisted Selective Autophagy Is Essential for Muscle Maintenance

(2010) Verena Arndt et al.   CURRENT BIOLOGY

Dynamic processes that reflect anti-apoptotic strategies set up by HspB1 (Hsp27)

(2010) Catherine Paul et al.   EXPERIMENTAL CELL RESEARCH

Independent evolution of the core domain and its flanking sequences in small heat shock proteins

(2010) Thomas Kriehuber et al.   FASEB JOURNAL

Mutant HSPB8 causes motor neuron-specific neurite degeneration

(2010) Joy Irobi et al.   HUMAN MOLECULAR GENETICS

HSPB7 is the most potent polyQ aggregation suppressor within the HSPB family of molecular chaperones

(2010) Michel J. Vos et al.   HUMAN MOLECULAR GENETICS

Molecular chaperone-mediated rescue of mitophagy by a Parkin RING1 domain mutant

(2010) Johanna M. Rose 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

Involvement of Reductive Stress in the Cardiomyopathy in Transgenic Mice With Cardiac-Specific Overexpression of Heat Shock Protein 27

(2010) Xia Zhang et al.   HYPERTENSION

Increased Monomerization of Mutant HSPB1 Leads to Protein Hyperactivity in Charcot-Marie-Tooth Neuropathy

(2010) Leonardo Almeida-Souza et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

DNAJB6 Induces Degradation of β-Catenin and Causes Partial Reversal of Mesenchymal Phenotype

(2010) Aparna Mitra et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Identification of theDrosophilaOrtholog of HSPB8

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

Role of DnaJ G/F-rich Domain in Conformational Recognition and Binding of Protein Substrates

(2010) Judit Perales-Calvo et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

BAG-6 is essential for selective elimination of defective proteasomal substrates

(2010) Ryosuke Minami et al.   JOURNAL OF CELL BIOLOGY

Characterization of BAG3 cleavage during apoptosis of pancreatic cancer cells

(2010) Hua-Qin Wang et al.   JOURNAL OF CELLULAR PHYSIOLOGY

Selective degradation of aggregate-prone CryAB mutants by HSPB1 is mediated by ubiquitin–proteasome pathways

(2010) Huali Zhang et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

Map3k1, Il6st, Gzmk, and Hspb3 gene coexpression network in the mechanism of freezing reaction in mice

(2010) Elena M. Kondaurova et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Differential expression and induction of small heat shock proteins in rat brain and cultured hippocampal neurons

(2010) Britta Bartelt Kirbach et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Doxycycline Attenuates Protein Aggregation in Cardiomyocytes and Improves Survival of a Mouse Model of Cardiac Proteinopathy

(2010) Hanqiao Zheng et al.   JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

A novel HSPB1 mutation in an Italian patient with CMT2/dHMN phenotype

(2010) M. Luigetti et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

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

(2010) Jurre Hageman et al.   MOLECULAR CELL

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

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

Mutant small heat shock protein B3 causes motor neuropathy: Utility of a candidate gene approach

(2010) S. J. Kolb et al.   NEUROLOGY

The p.G154S mutation of the alpha-B crystallin gene (CRYAB) causes late-onset distal myopathy

(2010) Peter Reilich et al.   NEUROMUSCULAR DISORDERS

Inheritance patterns and phenotypic features of myofibrillar myopathy associated with a BAG3 mutation

(2010) Zagaa Odgerel et al.   NEUROMUSCULAR DISORDERS

Infantile onset myofibrillar myopathy due to recessive CRYAB mutations

(2010) Katharine M.L. Forrest et al.   NEUROMUSCULAR DISORDERS

Dynamic Subunit Exchange and the Regulation of Microtubule Assembly by the Stress Response Protein Human αB Crystallin

(2010) Scott A. Houck et al.   PLoS One

Peripheral Protein Quality Control Removes Unfolded CFTR from the Plasma Membrane

(2010) T. Okiyoneda et al.   SCIENCE

Differential involvement of sarcomeric proteins in myofibrillar myopathies: a morphological and immunohistochemical study

(2009) Kristl G. Claeys et al.   ACTA NEUROPATHOLOGICA

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

(2009) Margit Fuchs et al.   BIOCHEMICAL JOURNAL

Thermally induced structural changes of intrinsically disordered small heat shock protein Hsp22

(2009) Alexey S. Kazakov et al.   BIOPHYSICAL CHEMISTRY

Myofibrillar Myopathies: A Clinical and Myopathological Guide

(2009) Rolf Schröder et al.   BRAIN PATHOLOGY

Why proteins without an α-crystallin domain should not be included in the human small heat shock protein family HSPB

(2009) Guido Kappé et al.   CELL STRESS & CHAPERONES

Biochemical and Mechanical Dysfunction in a Mouse Model of Desmin-Related Myopathy

(2009) Alina Maloyan et al.   CIRCULATION RESEARCH

Neural stem cell self-renewal requires the Mrj co-chaperone

(2009) Erica D. Watson et al.   DEVELOPMENTAL DYNAMICS

Protein quality control during aging involves recruitment of the macroautophagy pathway by BAG3

(2009) Martin Gamerdinger et al.   EMBO JOURNAL

The ataxia protein sacsin is a functional co-chaperone that protects against polyglutamine-expanded ataxin-1

(2009) David A. Parfitt et al.   HUMAN MOLECULAR GENETICS

Single Molecule Force Spectroscopy of the Cardiac Titin N2B Element

(2009) Yi Zhu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Phosphorylation Dependence of Hsp27 Multimeric Size and Molecular Chaperone Function

(2009) David Hayes et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Autoregulation of co-chaperone BAG3 gene transcription

(2009) Antonio Gentilella et al.   JOURNAL OF CELLULAR BIOCHEMISTRY

The Small Heat-Shock Proteins HSPB2 and HSPB3 Form Well-defined Heterooligomers in a Unique 3 to 1 Subunit Ratio

(2009) John den Engelsman et al.   JOURNAL OF MOLECULAR BIOLOGY

Crystal Structures of α-Crystallin Domain Dimers of αB-Crystallin and Hsp20

(2009) C. Bagnéris et al.   JOURNAL OF MOLECULAR BIOLOGY

Novel Subtype-Specific Genes Identify Distinct Subpopulations of Callosal Projection Neurons

(2009) B. J. Molyneaux et al.   JOURNAL OF NEUROSCIENCE

Substrate binding site flexibility of the small heat shock protein molecular chaperones

(2009) N. Jaya et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Bis deficiency results in early lethality with metabolic deterioration and involution of spleen and thymus

(2008) Dong-Ye Youn et al.   AMERICAN JOURNAL OF PHYSIOLOGY-ENDOCRINOLOGY AND METABOLISM

Gene expression profiling in human skeletal muscle during recovery from eccentric exercise

(2008) D. J. Mahoney et al.   AMERICAN JOURNAL OF PHYSIOLOGY-REGULATORY INTEGRATIVE AND COMPARATIVE PHYSIOLOGY

Mutation in BAG3 causes severe dominant childhood muscular dystrophy

(2008) Duygu Selcen et al.   ANNALS OF NEUROLOGY

The Hsp40 family chaperone protein DnaJB6 enhances Schlafen1 nuclear localization which is critical for promotion of cell-cycle arrest in T-cells

(2008) Yafeng Zhang et al.   BIOCHEMICAL JOURNAL

Abnormal Assemblies and Subunit Exchange of αB-Crystallin R120 Mutants Could Be Associated with Destabilization of the Dimeric Substructure†

(2008) Magalie Michiel et al.   BIOCHEMISTRY

Guidelines for the nomenclature of the human heat shock proteins

(2008) Harm H. Kampinga et al.   CELL STRESS & CHAPERONES

HspB8 Participates in Protein Quality Control by a Non-chaperone-like Mechanism That Requires eIF2α Phosphorylation

(2008) Serena Carra et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Truncation of αB-Crystallin by the Myopathy-causing Q151X Mutation Significantly Destabilizes the Protein Leading to Aggregate Formation in Transfected Cells

(2008) Victoria H. Hayes et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Small Heat Shock Protein Activity Is Regulated by Variable Oligomeric Substructure

(2008) Justin L. P. Benesch et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

bag3 gene expression is regulated by heat shock factor 1

(2008) Silvia Franceschelli et al.   JOURNAL OF CELLULAR PHYSIOLOGY

Proteasome inhibitor MG132 induces BAG3 expression through activation of heat shock factor 1

(2008) Zhen-Xian Du et al.   JOURNAL OF CELLULAR PHYSIOLOGY

A clinical phenotype of distal hereditary motor neuronopathy type II with a novel HSPB1 mutation

(2008) Yoshihisa Ikeda et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Mutations in the HSP27 (HSPB1) gene cause dominant, recessive, and sporadic distal HMN/CMT type 2

(2008) H. Houlden et al.   NEUROLOGY

Electron microscopy in myofibrillar myopathies reveals clues to the mutated gene

(2008) K.G. Claeys et al.   NEUROMUSCULAR DISORDERS

Global expression profiling identifies a novel biosignature for protein aggregation R120GCryAB cardiomyopathy in mice

(2008) Namakkal S. Rajasekaran et al.   PHYSIOLOGICAL GENOMICS

Autophagy is an adaptive response in desmin-related cardiomyopathy

(2008) P. Tannous et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Non-pathogenic protein aggregates in skeletal muscle in MLF1 transgenic mice

(2007) Zhi-Fang Li et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES