Muscle expression of mutant androgen receptor accounts for systemic and motor neuron disease phenotypes in spinal and bulbar muscular atrophy
about
Autophagy in polyglutamine disease: Imposing order on disorder or contributing to the chaos?Ataxin-2 regulates RGS8 translation in a new BAC-SCA2 transgenic mouse modelLarge Polyglutamine Repeats Cause Muscle Degeneration in SCA17 Mice.The polyglutamine-expanded androgen receptor responsible for spinal and bulbar muscular atrophy inhibits the APC/C(Cdh1) ubiquitin ligase complex.Peripheral androgen receptor gene suppression rescues disease in mouse models of spinal and bulbar muscular atrophy.Disrupting SUMOylation enhances transcriptional function and ameliorates polyglutamine androgen receptor-mediated disease.Increased mitophagy in the skeletal muscle of spinal and bulbar muscular atrophy patientsPeripheral huntingtin silencing does not ameliorate central signs of disease in the B6.HttQ111/+ mouse model of Huntington's diseaseIdentification of neuron selective androgen receptor inhibitors.The Role of the Heat Shock Protein B8 (HSPB8) in Motoneuron Diseases.The C. elegans VAPB homolog VPR-1 is a permissive signal for gonad development.Muscle matters in Kennedy's disease.Polyglutamine-expanded androgen receptor interferes with TFEB to elicit autophagy defects in SBMA.Spinal and Bulbar Muscular Atrophy.Synergic prodegradative activity of Bicalutamide and trehalose on the mutant androgen receptor responsible for spinal and bulbar muscular atrophy.Transcriptional profile of muscle following acute induction of symptoms in a mouse model of Kennedy's disease/spinobulbar muscular atrophy.Contractile dysfunction in muscle may underlie androgen-dependent motor dysfunction in spinal bulbar muscular atrophy.Human adipose-derived mesenchymal stem cells as a new model of spinal and bulbar muscular atrophy.Androgen-dependent loss of muscle BDNF mRNA in two mouse models of SBMA.Nemo-like kinase is a novel regulator of spinal and bulbar muscular atrophy.Aberrant Autophagic Response in The Muscle of A Knock-in Mouse Model of Spinal and Bulbar Muscular Atrophy.Transcriptional induction of the heat shock protein B8 mediates the clearance of misfolded proteins responsible for motor neuron diseases.Defects in Neuromuscular Transmission May Underlie Motor Dysfunction in Spinal and Bulbar Muscular AtrophyMiR-298 Counteracts Mutant Androgen Receptor Toxicity in Spinal and Bulbar Muscular Atrophy.Selective Expression of Osteopontin in ALS-resistant Motor Neurons is a Critical Determinant of Late Phase Neurodegeneration Mediated by Matrix Metalloproteinase-9.Glycolytic-to-oxidative fiber-type switch and mTOR signaling activation are early-onset features of SBMA muscle modified by high-fat dietNon-neural phenotype of spinal and bulbar muscular atrophy: results from a large cohort of Italian patients.Rescue of Metabolic Alterations in AR113Q Skeletal Muscle by Peripheral Androgen Receptor Gene Silencing.Spinal and Bulbar Muscular Atrophy Overview.Beta-agonist stimulation ameliorates the phenotype of spinal and bulbar muscular atrophy mice and patient-derived myotubes.Potential therapeutic targets in polyglutamine-mediated diseases.Motor neuron degeneration in spinal and Bulbar Muscular Atrophy is a skeletal muscle-driven process: Relevance to therapy development and implications for related motor neuron diseases.Diagnostic Clinical, Electrodiagnostic and Muscle Pathology Features of Spinal and Bulbar Muscular Atrophy.The Role of the Protein Quality Control System in SBMA.Targeted Molecular Therapies for SBMA.Exercise Therapy in Spinobulbar Muscular Atrophy and Other Neuromuscular Disorders.In Vitro and In Vivo Modeling of Spinal and Bulbar Muscular Atrophy.The Role of Skeletal Muscle in Amyotrophic Lateral Sclerosis.Polyglutamine androgen receptor-mediated neuromuscular disease.Inhibition of retrograde transport modulates misfolded protein accumulation and clearance in motoneuron diseases.
P2860
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P2860
Muscle expression of mutant androgen receptor accounts for systemic and motor neuron disease phenotypes in spinal and bulbar muscular atrophy
description
2014 nî lūn-bûn
@nan
2014 թուականի Ապրիլին հրատարակուած գիտական յօդուած
@hyw
2014 թվականի ապրիլին հրատարակված գիտական հոդված
@hy
2014年の論文
@ja
2014年論文
@yue
2014年論文
@zh-hant
2014年論文
@zh-hk
2014年論文
@zh-mo
2014年論文
@zh-tw
2014年论文
@wuu
name
Muscle expression of mutant an ...... al and bulbar muscular atrophy
@ast
Muscle expression of mutant an ...... al and bulbar muscular atrophy
@en
type
label
Muscle expression of mutant an ...... al and bulbar muscular atrophy
@ast
Muscle expression of mutant an ...... al and bulbar muscular atrophy
@en
prefLabel
Muscle expression of mutant an ...... al and bulbar muscular atrophy
@ast
Muscle expression of mutant an ...... al and bulbar muscular atrophy
@en
P2093
P2860
P1433
P1476
Muscle expression of mutant an ...... al and bulbar muscular atrophy
@en
P2093
Albert R La Spada
Bryce L Sopher
C Frank Bennett
Constanza J Cortes
Edith Lopez
G Diane Shelton
Ling T Guo
Seiya Tokunaga
P2860
P304
P356
10.1016/J.NEURON.2014.03.001
P407
P577
2014-04-01T00:00:00Z