Androgen receptor polyglutamine expansion drives age-dependent quality control defects and muscle dysfunction

Selective modulation of the androgen receptor AF2 domain rescues degeneration in spinal bulbar muscular atrophy

(2018) Nisha M Badders et al.   NATURE MEDICINE

Ubiquitinated proteins promote the association of proteasomes with the deubiquitinating enzyme Usp14 and the ubiquitin ligase Ube3c

(2017) Chueh-Ling Kuo et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The Ubiquitination, Disaggregation and Proteasomal Degradation Machineries in Polyglutamine Disease

(2017) Samir R. Nath et al.   Frontiers in Molecular 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

UBQLN2 Mediates Autophagy-Independent Protein Aggregate Clearance by the Proteasome

(2016) Roland Hjerpe et al.   CELL

Polyglutamine androgen receptor-mediated neuromuscular disease

(2016) Elisa Giorgetti et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Reply to Vangala et al. : Complete inhibition of the proteasome reduces new proteasome production by causing Nrf1 aggregation

(2016) Zhe Sha et al.   CURRENT BIOLOGY

A small-molecule Nrf1 and Nrf2 activator mitigates polyglutamine toxicity in spinal and bulbar muscular atrophy

(2016) Laura C. Bott et al.   HUMAN MOLECULAR GENETICS

Rescue of Metabolic Alterations in AR113Q Skeletal Muscle by Peripheral Androgen Receptor Gene Silencing

(2016) Elisa Giorgetti et al.   Cell Reports

Proteasome dysfunction triggers activation of SKN-1A/Nrf1 by the aspartic protease DDI-1

(2016) Nicolas J Lehrbach et al.   eLife

The aspartyl protease DDI2 activates Nrf1 to compensate for proteasome dysfunction

(2016) Shun Koizumi et al.   eLife

Targeting Hsp90/Hsp70-Based Protein Quality Control for Treatment of Adult Onset Neurodegenerative Diseases

(2015) William B. Pratt et al.   Annual Review of Pharmacology and Toxicology

DnaJ-1 and karyopherin α3 suppress degeneration in a newDrosophilamodel of Spinocerebellar Ataxia Type 6

(2015) Wei-Ling Tsou et al.   HUMAN MOLECULAR GENETICS

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

Tau-driven 26S proteasome impairment and cognitive dysfunction can be prevented early in disease by activating cAMP-PKA signaling

(2015) Natura Myeku et al.   NATURE MEDICINE

The deubiquitinase ataxin-3 requires Rad23 and DnaJ-1 for its neuroprotective role in Drosophila melanogaster

(2015) Wei-Ling Tsou et al.   NEUROBIOLOGY OF DISEASE

cAMP-induced phosphorylation of 26S proteasomes on Rpn6/PSMD11 enhances their activity and the degradation of misfolded proteins

(2015) Sudarsanareddy Lokireddy et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

c-Abl Regulates Proteasome Abundance by Controlling the Ubiquitin-Proteasomal Degradation of PSMA7 Subunit

(2015) Dapei Li et al.   Cell Reports

Autoubiquitination of the 26S Proteasome on Rpn13 Regulates Breakdown of Ubiquitin Conjugates

(2014) H. C. Besche et al.   EMBO JOURNAL

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

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

Ubiquitin-binding site 2 of ataxin-3 prevents its proteasomal degradation by interacting with Rad23

(2014) Jessica R. Blount et al.   Nature Communications

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

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

An optimal ubiquitin-proteasome pathway in the nervous system: the role of deubiquitinating enzymes

(2014) Gorica Ristic et al.   Frontiers in Molecular Neuroscience

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

(2013) Adriana Malena et al.   ACTA NEUROPATHOLOGICA

A bis-Benzylidine Piperidone Targeting Proteasome Ubiquitin Receptor RPN13/ADRM1 as a Therapy for Cancer

(2013) Ravi K. Anchoori et al.   CANCER CELL

Activity-Based Imaging Probes of the Proteasome

(2013) Kimberly Cornish Carmony et al.   CELL BIOCHEMISTRY AND BIOPHYSICS

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

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

Activation of Hsp70 reduces neurotoxicity by promoting polyglutamine protein degradation

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

Impaired motoneuronal retrograde transport in two models of SBMA implicates two sites of androgen action

(2011) Michael Q. Kemp et al.   HUMAN MOLECULAR GENETICS

Basic Leucine Zipper Protein Cnc-C Is a Substrate and Transcriptional Regulator of the Drosophila 26S Proteasome

(2010) K. B. Grimberg et al.   MOLECULAR AND CELLULAR BIOLOGY

Proteasomal Degradation Is Transcriptionally Controlled by TCF11 via an ERAD-Dependent Feedback Loop

(2010) Janos Steffen et al.   MOLECULAR CELL

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

Clinical features of spinal and bulbar muscular atrophy

(2009) Lindsay E. Rhodes et al.   BRAIN

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

Chemical Manipulation of Hsp70 ATPase Activity Regulates Tau Stability

(2009) U. K. Jinwal et al.   JOURNAL OF NEUROSCIENCE

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

(2009) Isabella Palazzolo et al.   NEURON

17-DMAG ameliorates polyglutamine-mediated motor neuron degeneration through well-preserved proteasome function in an SBMA model mouse

(2008) Keisuke Tokui et al.   HUMAN MOLECULAR GENETICS

Mitochondrial abnormalities in spinal and bulbar muscular atrophy

(2008) Srikanth Ranganathan et al.   HUMAN MOLECULAR GENETICS

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

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