Beta-agonist stimulation ameliorates the phenotype of spinal and bulbar muscular atrophy mice and patient-derived myotubes

Spinal and bulbar muscular atrophy (SBMA) is a neuromuscular disease characterized by the loss of lower motor neurons. SBMA is caused by expansions of a polyglutamine tract in the gene coding for androgen receptor (AR). Expression of polyglutamine-expanded AR causes damage to motor neurons and skele...

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Veröffentlicht in:	Scientific reports 2017-01, Vol.7 (1), p.41046, Article 41046
Hauptverfasser:	Milioto, Carmelo, Malena, Adriana, Maino, Eleonora, Polanco, Maria J., Marchioretti, Caterina, Borgia, Doriana, Pereira, Marcelo Gomes, Blaauw, Bert, Lieberman, Andrew P., Venturini, Roberta, Plebani, Mario, Sambataro, Fabio, Vergani, Lodovica, Pegoraro, Elena, Sorarù, Gianni, Pennuto, Maria
Format:	Artikel
Sprache:	eng
Schlagworte:	1-Phosphatidylinositol 3-kinase 13/1 13/106 13/109 13/95 14 14/63 631/378/1689/364 692/308/2778 96 96/1 Adrenergic beta-Agonists - pharmacology AKT protein Androgen receptors Animals Atrophy Clenbuterol Clenbuterol - pharmacology Disease Models, Animal Fibers Glycolysis Humanities and Social Sciences Humans Hypertrophy Male Mice Mice, Transgenic Motor neurons Motor task performance multidisciplinary Muscle Fibers, Skeletal - cytology Muscle Fibers, Skeletal - drug effects Muscle Fibers, Skeletal - metabolism Muscles Muscular Disorders, Atrophic - drug therapy Muscular Disorders, Atrophic - metabolism Muscular Disorders, Atrophic - pathology Myotubes Neural coding Peptides Phosphatidylinositol 3-Kinases - metabolism Polyglutamine Proto-Oncogene Proteins c-akt - metabolism Rapamycin Receptors, Androgen - genetics Rodents Science Science (multidisciplinary) Signal Transduction Skeletal muscle Spinal and bulbar muscular atrophy TOR protein TOR Serine-Threonine Kinases - metabolism Trinucleotide repeat diseases Trinucleotide Repeat Expansion
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