The Role of the Protein Quality Control System in SBMA

Proteasome-mediated Proteolysis of the Polyglutamine-expanded Androgen Receptor Is a Late Event in Spinal and Bulbar Muscular Atrophy (SBMA) Pathogenesis

(2015) Erin M. Heine et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

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

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

Endoplasmic reticulum stress in spinal and bulbar muscular atrophy: a potential target for therapy

(2014) Karli Montague et al.   BRAIN

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

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

A model in which heat shock protein 90 targets protein-folding clefts: Rationale for a new approach to neuroprotective treatment of protein folding diseases

(2014) William B Pratt et al.   EXPERIMENTAL BIOLOGY AND MEDICINE

Paeoniflorin eliminates a mutant AR via NF-YA-dependent proteolysis in spinal and bulbar muscular atrophy

(2014) Genki Tohnai et al.   HUMAN MOLECULAR GENETICS

Pioglitazone suppresses neuronal and muscular degeneration caused by polyglutamine-expanded androgen receptors

(2014) Madoka Iida et al.   HUMAN MOLECULAR GENETICS

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

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

(2014) Constanza J Cortes et al.   NATURE NEUROSCIENCE

Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases

(2014) Claudio Hetz et al.   NATURE REVIEWS NEUROSCIENCE

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

(2014) Mariarita Galbiati et al.   NEUROCHEMISTRY INTERNATIONAL

Correlation of clinical and molecular features in spinal bulbar muscular atrophy

(2014) P. Fratta et al.   NEUROLOGY

Early onset and novel features in a spinal and bulbar muscular atrophy patient with a 68 CAG repeat

(2014) Christopher Grunseich et al.   NEUROMUSCULAR DISORDERS

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

Muscle Matters in Kennedy’s Disease

(2014) Carlo Rinaldi et al.   NEURON

Human Adipose-Derived Mesenchymal Stem Cells as a New Model of Spinal and Bulbar Muscular Atrophy

(2014) Marta Dossena et al.   PLoS One

Proteostasis impairment in protein-misfolding and -aggregation diseases

(2014) Mark S. Hipp et al.   TRENDS IN CELL BIOLOGY

The ER mitochondria calcium cycle and ER stress response as therapeutic targets in amyotrophic lateral sclerosis

(2014) Vedrana Tadic et al.   Frontiers in Cellular Neuroscience

The heat-shock response co-inducer arimoclomol protects against retinal degeneration in rhodopsin retinitis pigmentosa

(2014) D A Parfitt et al.   Cell Death & Disease

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

Co-induction of the heat shock response ameliorates disease progression in a mouse model of human spinal and bulbar muscular atrophy: implications for therapy

(2013) Bilal Malik et al.   BRAIN

Update in inclusion body myositis

(2013) Pedro Machado et al.   CURRENT OPINION IN RHEUMATOLOGY

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

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

p62/SQSTM1 Differentially Removes the Toxic Mutant Androgen Receptor via Autophagy and Inclusion Formation in a Spinal and Bulbar Muscular Atrophy Mouse Model

(2013) H. Doi et al.   JOURNAL OF NEUROSCIENCE

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

(2013) Paola Rusmini et al.   NEUROBIOLOGY OF AGING

Sequencing analysis of the spinal bulbar muscular atrophy CAG expansion reveals absence of repeat interruptions

(2013) Pietro Fratta et al.   NEUROBIOLOGY OF AGING

Pharmacological reduction of ER stress protects against TDP-43 neuronal toxicity in vivo

(2013) Alexandra Vaccaro et al.   NEUROBIOLOGY OF DISEASE

Spinal and bulbar muscular atrophy: pathogenesis and clinical management

(2013) C Grunseich et al.   ORAL DISEASES

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

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

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

Heat shock factor-1 influences pathological lesion distribution of polyglutamine-induced neurodegeneration

(2013) Naohide Kondo et al.   Nature Communications

More than a bystander: the contributions of intrinsic skeletal muscle defects in motor neuron diseases

(2013) Justin G. Boyer et al.   Frontiers in Physiology

Mechanisms Mediating Spinal and Bulbar Muscular Atrophy: Investigations into Polyglutamine-Expanded Androgen Receptor Function and Dysfunction

(2013) Lenore K. Beitel et al.   Frontiers in Neurology

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

(2013) Justin G Boyer et al.   Skeletal Muscle

Guidelines for the use and interpretation of assays for monitoring autophagy

(2012) Daniel J. Klionsky et al.   Autophagy

Development and therapeutic impact of HDAC6-selective inhibitors

(2012) Sabrina Dallavalle et al.   BIOCHEMICAL PHARMACOLOGY

Toxic and non-toxic aggregates from the SBMA and normal forms of androgen receptor have distinct oligomeric structures

(2012) Tobias Jochum et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Role of androgen receptor polyQ chain elongation in Kennedy's disease and use of natural osmolytes as potential therapeutic targets

(2012) Raj Kumar   IUBMB LIFE

Accumulation of Toxic  -Synuclein Oligomer within Endoplasmic Reticulum Occurs in  -Synucleinopathy In Vivo

(2012) E. Colla et al.   JOURNAL OF NEUROSCIENCE

Endoplasmic Reticulum Stress Is Important for the Manifestations of  -Synucleinopathy In Vivo

(2012) E. Colla et al.   JOURNAL OF NEUROSCIENCE

Activation of Hsp70 reduces neurotoxicity by promoting polyglutamine protein degradation

(2012) Adrienne M Wang et al.   Nature Chemical Biology

Developing treatment for spinal and bulbar muscular atrophy

(2012) Kenneth H. Fischbeck   PROGRESS IN NEUROBIOLOGY

HDAC6 at the Intersection of Neuroprotection and Neurodegeneration

(2012) Constantin d'Ydewalle et al.   TRAFFIC

TFEB regulates autophagy: An integrated coordination of cellular degradation and recycling processes

(2011) Carmine Settembre et al.   Autophagy

Absence of disturbed axonal transport in spinal and bulbar muscular atrophy

(2011) B. Malik et al.   HUMAN MOLECULAR GENETICS

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

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

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

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

(2011) Elisa Onesto et al.   JOURNAL OF NEUROCHEMISTRY

SIRT1 Modulates Aggregation and Toxicity through Deacetylation of the Androgen Receptor in Cell Models of SBMA

(2011) H. L. Montie et al.   JOURNAL OF NEUROSCIENCE

Vascular endothelial growth factor ameliorates the ataxic phenotype in a mouse model of spinocerebellar ataxia type 1

(2011) Marija Cvetanovic et al.   NATURE MEDICINE

Alteration of protein folding and degradation in motor neuron diseases: Implications and protective functions of small heat shock proteins

(2011) Serena Carra et al.   PROGRESS IN NEUROBIOLOGY

Macroautophagy Is Regulated by the UPR–Mediator CHOP and Accentuates the Phenotype of SBMA Mice

(2011) Zhigang Yu et al.   PLoS Genetics

A role of small heat shock protein B8 (HspB8) in the autophagic removal of misfolded proteins responsible for neurodegenerative diseases

(2010) Valeria Crippa et al.   Autophagy

Chaperone-Assisted Selective Autophagy Is Essential for Muscle Maintenance

(2010) Verena Arndt et al.   CURRENT BIOLOGY

HDAC6 controls autophagosome maturation essential for ubiquitin-selective quality-control autophagy

(2010) Joo-Yong Lee et al.   EMBO JOURNAL

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

An Interdomain Interaction of the Androgen Receptor Is Required for Its Aggregation and Toxicity in Spinal and Bulbar Muscular Atrophy

(2010) Christopher R. Orr et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Pathogenic Lysosomal Depletion in Parkinson's Disease

(2010) B. Dehay et al.   JOURNAL OF NEUROSCIENCE

Transcription Factor Nrf1 Mediates the Proteasome Recovery Pathway after Proteasome Inhibition in Mammalian Cells

(2010) Senthil K. Radhakrishnan et al.   MOLECULAR CELL

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

(2010) Jurre Hageman et al.   MOLECULAR CELL

Enhancement of proteasome activity by a small-molecule inhibitor of USP14

(2010) Byung-Hoon Lee 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

17-AAG increases autophagic removal of mutant androgen receptor in spinal and bulbar muscular atrophy

(2010) Paola Rusmini et al.   NEUROBIOLOGY OF DISEASE

Native Functions of the Androgen Receptor Are Essential to Pathogenesis in a Drosophila Model of Spinobulbar Muscular Atrophy

(2010) Natalia B. Nedelsky et al.   NEURON

Therapeutic approaches to spinal and bulbar muscular atrophy

(2010) Srikanth Ranganathan et al.   TRENDS IN PHARMACOLOGICAL SCIENCES

Autophagy and access:  Understanding the role of androgen receptor subcellular localization in SBMA

(2009) Heather Montie et al.   Autophagy

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

(2009) Serena Carra   Autophagy

Microtubule-dependent formation of the stigmoid body as a cytoplasmic inclusion distinct from pathological aggresomes

(2009) Ryutaro Fujinaga et al.   HISTOCHEMISTRY AND CELL BIOLOGY

Cytoplasmic retention of polyglutamine-expanded androgen receptor ameliorates disease via autophagy in a mouse model of spinal and bulbar muscular atrophy

(2009) Heather L. Montie et al.   HUMAN MOLECULAR GENETICS

Heat shock proteins in neurodegenerative diseases: Pathogenic roles and therapeutic implications

(2009) Hiroaki Adachi et al.   INTERNATIONAL JOURNAL OF HYPERTHERMIA

Hypoxia-Inducible mir-210 Regulates Normoxic Gene Expression Involved in Tumor Initiation

(2009) Xin Huang et al.   MOLECULAR CELL

A role for motoneuron subtype–selective ER stress in disease manifestations of FALS mice

(2009) Smita Saxena et al.   NATURE NEUROSCIENCE

Living or dying in three quarter time: Neonatal orchestration of hippocampal cell death pathways by androgens and excitatory GABA

(2008) C.D. Foradori et al.   EXPERIMENTAL NEUROLOGY

Late stage treatment with arimoclomol delays disease progression and prevents protein aggregation in the SOD1G93A mouse model of ALS

(2008) Bernadett Kalmar et al.   JOURNAL OF NEUROCHEMISTRY

Nrf2 Activation in Astrocytes Protects against Neurodegeneration in Mouse Models of Familial Amyotrophic Lateral Sclerosis

(2008) M. R. Vargas et al.   JOURNAL OF NEUROSCIENCE

Arimoclomol at dosages up to 300 mg/day is well tolerated and safe in amyotrophic lateral sclerosis

(2008) Merit E. Cudkowicz et al.   MUSCLE & NERVE

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

(2008) Georgia Anagnostou et al.   NEUROBIOLOGY OF AGING

Adapting Proteostasis for Disease Intervention

(2008) William E. Balch et al.   SCIENCE

The role of the polyglutamine tract in androgen receptor

(2007) Isabella Palazzolo et al.   JOURNAL OF STEROID BIOCHEMISTRY AND MOLECULAR BIOLOGY

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

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