Muscle expression of mutant androgen receptor accounts for systemic and motor neuron disease phenotypes in spinal and bulbar muscular atrophy - Wikidata - wikimedia - TriplyDB

Muscle expression of mutant androgen receptor accounts for systemic and motor neuron disease phenotypes in spinal and bulbar muscular atrophy

Muscle expression of mutant androgen receptor accounts for systemic and motor neuron disease phenotypes in spinal and bulbar muscular atrophy

http://www.wikidata.org/entity/Q33894661

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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 model Large 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 patients Peripheral huntingtin silencing does not ameliorate central signs of disease in the B6.HttQ111/+ mouse model of Huntington's disease Identification 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 Atrophy MiR-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 diet Non-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.

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Muscle expression of mutant androgen receptor accounts for systemic and motor neuron disease phenotypes in spinal and bulbar muscular atrophy

http://www.wikidata.org/entity/Q33894661

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2014 nî lūn-bûn

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2014 թուականի Ապրիլին հրատարակուած գիտական յօդուած

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2014 թվականի ապրիլին հրատարակված գիտական հոդված

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2014年の論文

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2014年論文

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2014年論文

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2014年論文

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2014年論文

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2014年论文

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Muscle expression of mutant an ...... al and bulbar muscular atrophy

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Muscle expression of mutant an ...... al and bulbar muscular atrophy

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J.NEURON.2014.03.001

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Muscle expression of mutant an ...... al and bulbar muscular atrophy

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Albert R La Spada

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Bryce L Sopher

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C Frank Bennett

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Constanza J Cortes

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Edith Lopez

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G Diane Shelton

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Gene Hung

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Linda Ly

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Ling T Guo

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Seiya Tokunaga

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P2860

Motor neuron involvement in multisystem proteinopathy: implications for ALS

Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS

A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD

Androgen-dependent pathology demonstrates myopathic contribution to the Kennedy disease phenotype in a mouse knock-in model

Identification and characterization of a spinal muscular atrophy-determining gene

Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis

Onset and progression in inherited ALS determined by motor neurons and microglia

Peripheral androgen receptor gene suppression rescues disease in mouse models of spinal and bulbar muscular atrophy.

Overexpression of wild-type androgen receptor in muscle recapitulates polyglutamine disease.

ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43.

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295-307

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10.1016/J.NEURON.2014.03.001

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Don W Cleveland

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261756130

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4096235

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