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The ubiquitin-proteasome system in neurodegenerative diseases: precipitating factor, yet part of the solution

Frontiers in molecular neuroscience, 2014-07, Vol.7, p.70-70

2014

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

Autophagy, 2010-02, Vol.6 (2), p.217-227

2010

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

Human molecular genetics, 2016-05, Vol.25 (10), p.1979-1989

2016

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Muscle Matters in Kennedy’s Disease

Neuron (Cambridge, Mass.), 2014-04, Vol.82 (2), p.251-253

2014

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Shaping proteostasis at the cellular, tissue, and organismal level

The Journal of cell biology, 2017-05, Vol.216 (5), p.1231-1241

2017

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Stem cell-derived motor neurons from spinal and bulbar muscular atrophy patients

Neurobiology of disease, 2014-10, Vol.70, p.12-20

2014

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MiR-298 Counteracts Mutant Androgen Receptor Toxicity in Spinal and Bulbar Muscular Atrophy

Molecular therapy, 2016-05, Vol.24 (5), p.937-945

2016

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Hetero-oligomerization of TDP-43 carboxy-terminal fragments with cellular proteins contributes to proteotoxicity

Communications biology, 2024-06, Vol.7 (1), p.743-21, Article 743

2024

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Insulinlike growth factor (IGF)-1 administration ameliorates disease manifestations in a mouse model of spinal and bulbar muscular atrophy

Molecular medicine (Cambridge, Mass.), 2012, Vol.18 (9), p.1261-1268

2012

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Embryo integrity regulates maternal proteostasis and stress resilience

Genes & development, 2020-05, Vol.34 (9-10), p.678-687

2020

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Nuclear receptor signaling via NHR-49/MDT-15 regulates stress resilience and proteostasis in response to reproductive and metabolic cues

Genes & development, 2024-06, Vol.38 (9-10), p.380

2024

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A Conserved Unfoldase Activity for the p97 AAA-ATPase in Proteasomal Degradation

Journal of molecular biology, 2009-12, Vol.394 (4), p.732-746

2009

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Mutation in CPT1C Associated With Pure Autosomal Dominant Spastic Paraplegia

JAMA neurology, 2015-05, Vol.72 (5), p.561

2015

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

Neuromuscular disorders : NMD, 2014-11, Vol.24 (11), p.978-981

2014

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Systemic Delivery of MicroRNA Using Recombinant Adeno-associated Virus Serotype 9 to Treat Neuromuscular Diseases in Rodents

Journal of visualized experiments, 2018-08 (138)

2018

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The polyglutamine-expanded androgen receptor responsible for spinal and bulbar muscular atrophy inhibits the APC/CCdh1 ubiquitin ligase complex

Scientific reports, 2016-06, Vol.6 (1), p.27703, Article 27703

2016

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The polyglutamine-expanded androgen receptor responsible for spinal and bulbar muscular atrophy inhibits the APC/C(Cdh1) ubiquitin ligase complex

Scientific reports, 2016-06, Vol.6, p.27703-27703

2016

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The polyglutamine-expanded androgen receptor has increased DNA binding and reduced transcriptional activity

Biochemistry and biophysics reports, 2015-09, Vol.3, p.134-139

2015

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Sexual Reassignment Fails to Prevent Kennedy’s Disease

Journal of neuromuscular diseases, 2016-03, Vol.3 (1), p.121-125

2016

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Systemic Delivery of MicroRNA Using Recombinant Adeno-associated Virus Serotype 9 to Treat Neuromuscular Diseases in Rodents

Journal of visualized experiments, 2018-08 (138)

2018

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Spinal and Bulbar Muscular Atrophy : New Insights into the Disease Mechanism and Prospects for Pharmacological Therapy

Bott, Laura C

2015

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REST mediates androgen receptor actions on gene repression and predicts early recurrence of prostate cancer

Nucleic acids research, 2014, Vol.42 (2), p.999-1015

2014

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3D gravity modelling of Colorado and Claromecó basins: new evidences for the evolution of the southwestern margin of Gondwana

International journal of earth sciences : Geologische Rundschau, 2021-10, Vol.110 (7), p.2295-2313

2021

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The Human Tumor Atlas Network: Charting Tumor Transitions across Space and Time at Single-Cell Resolution

Cell, 2020-04, Vol.181 (2), p.236-249

2020

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The ubiquitin-proteasome system in neurodegenerative diseases: precipitating factor, yet part of the solution

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

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

Muscle Matters in Kennedy’s Disease

Shaping proteostasis at the cellular, tissue, and organismal level

Stem cell-derived motor neurons from spinal and bulbar muscular atrophy patients

MiR-298 Counteracts Mutant Androgen Receptor Toxicity in Spinal and Bulbar Muscular Atrophy

Hetero-oligomerization of TDP-43 carboxy-terminal fragments with cellular proteins contributes to proteotoxicity

Insulinlike growth factor (IGF)-1 administration ameliorates disease manifestations in a mouse model of spinal and bulbar muscular atrophy

Embryo integrity regulates maternal proteostasis and stress resilience

Nuclear receptor signaling via NHR-49/MDT-15 regulates stress resilience and proteostasis in response to reproductive and metabolic cues

A Conserved Unfoldase Activity for the p97 AAA-ATPase in Proteasomal Degradation

Mutation in CPT1C Associated With Pure Autosomal Dominant Spastic Paraplegia

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

Systemic Delivery of MicroRNA Using Recombinant Adeno-associated Virus Serotype 9 to Treat Neuromuscular Diseases in Rodents

The polyglutamine-expanded androgen receptor responsible for spinal and bulbar muscular atrophy inhibits the APC/CCdh1 ubiquitin ligase complex

The polyglutamine-expanded androgen receptor responsible for spinal and bulbar muscular atrophy inhibits the APC/C(Cdh1) ubiquitin ligase complex

Variants in ATP6V0A1 cause progressive myoclonus epilepsy and developmental and epileptic encephalopathy

The polyglutamine-expanded androgen receptor has increased DNA binding and reduced transcriptional activity

Sexual Reassignment Fails to Prevent Kennedy’s Disease

Systemic Delivery of MicroRNA Using Recombinant Adeno-associated Virus Serotype 9 to Treat Neuromuscular Diseases in Rodents

Spinal and Bulbar Muscular Atrophy : New Insights into the Disease Mechanism and Prospects for Pharmacological Therapy

REST mediates androgen receptor actions on gene repression and predicts early recurrence of prostate cancer

3D gravity modelling of Colorado and Claromecó basins: new evidences for the evolution of the southwestern margin of Gondwana

The Human Tumor Atlas Network: Charting Tumor Transitions across Space and Time at Single-Cell Resolution

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