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

The Androgen Receptor's CAG/Glutamine Tract in Mouse Models of Neurological Disease and Cancer

(2017) Andrew P. Lieberman et al.   JOURNAL OF ALZHEIMERS DISEASE

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

Disrupting SUMOylation enhances transcriptional function and ameliorates polyglutamine androgen receptor–mediated disease

(2015) Jason P. Chua et al.   JOURNAL OF CLINICAL INVESTIGATION

Distinct Etiological Roles for Myocytes and Motor Neurons in a Mouse Model of Kennedy's Disease/Spinobulbar Muscular Atrophy

(2015) F. Ramzan et al.   JOURNAL OF NEUROSCIENCE

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

(2015) Valeria Crippa et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

BAG3 induces the sequestration of proteasomal clients into cytoplasmic puncta

(2014) Melania Minoia et al.   Autophagy

Myopathic Involvement and Mitochondrial Pathology in Kennedy Disease and in Other Motor Neuron Diseases

(2014) D. Orsucci et al.   CURRENT MOLECULAR MEDICINE

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

Androgens affect muscle, motor neuron, and survival in a mouse model of SOD1-related amyotrophic lateral sclerosis

(2014) Tanya Aggarwal et al.   NEUROBIOLOGY OF AGING

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

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

(2014) Marta Dossena et al.   PLoS One

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

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

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

PGC-1  is a male-specific disease modifier of human and experimental amyotrophic lateral sclerosis

(2013) J. Eschbach et al.   HUMAN MOLECULAR GENETICS

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

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

Conditional Expression of TGF-β1 in Skeletal Muscles Causes Endomysial Fibrosis and Myofibers Atrophy

(2013) Jigna Narola et al.   PLoS One

Quantitative proteome analysis of age-related changes in mouse gastrocnemius muscle using mTRAQ

(2013) Chae Young Hwang et al.   PROTEOMICS

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

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

Cullin 3 as a novel target in diverse pathologies

(2013) Ana Cristina Andérica-Romero et al.   Redox Biology

The Androgen Receptor in Health and Disease

(2012) Takahiro Matsumoto et al.   Annual Review of Physiology

Decreased mRNA Expression of PGC-1α and PGC-1α-Regulated Factors in the SOD1G93AALS Mouse Model and in Human Sporadic ALS

(2012) Nadine Thau et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

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

The anabolic/androgenic steroid nandrolone exacerbates gene expression modifications induced by mutant SOD1 in muscles of mice models of amyotrophic lateral sclerosis

(2011) Mariarita Galbiati et al.   PHARMACOLOGICAL RESEARCH

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

Kelch-like homologue 9 mutation is associated with an early onset autosomal dominant distal myopathy

(2010) Sebahattin Cirak et al.   BRAIN

Chaperone-Assisted Selective Autophagy Is Essential for Muscle Maintenance

(2010) Verena Arndt et al.   CURRENT BIOLOGY

Overexpression of Androgen Receptors in Target Musculature Confers Androgen Sensitivity to Motoneuron Dendrites

(2010) Anna L. Huguenard et al.   ENDOCRINOLOGY

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

Disrupted Transforming Growth Factor-  Signaling in Spinal and Bulbar Muscular Atrophy

(2010) M. Katsuno et al.   JOURNAL OF NEUROSCIENCE

Pathogenic Lysosomal Depletion in Parkinson's Disease

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

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

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

(2010) S. J. Kolb et al.   NEUROLOGY

Microarray Analysis of Gene Expression by Skeletal Muscle of Three Mouse Models of Kennedy Disease/Spinal Bulbar Muscular Atrophy

(2010) Kaiguo Mo et al.   PLoS One

Altered RNA splicing contributes to skeletal muscle pathology in Kennedy disease knock-in mice

(2009) Z. Yu et al.   Disease Models & Mechanisms

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

(2009) Martin Gamerdinger et al.   EMBO JOURNAL

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

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

Recovery of function in a myogenic mouse model of spinal bulbar muscular atrophy

(2009) Jamie A. Johansen et al.   NEUROBIOLOGY OF DISEASE

Overexpression of IGF-1 in Muscle Attenuates Disease in a Mouse Model of Spinal and Bulbar Muscular Atrophy

(2009) Isabella Palazzolo et al.   NEURON

Spinal and bulbar muscular atrophy: a motoneuron or muscle disease?

(2008) C JORDAN et al.   CURRENT OPINION IN PHARMACOLOGY