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We are analyzing https://link.springer.com/article/10.1007/s00401-013-1122-9.

Title:
Androgen-dependent impairment of myogenesis in spinal and bulbar muscular atrophy | Acta Neuropathologica
Description:
Spinal and bulbar muscular atrophy (SBMA) is an inherited neuromuscular disease caused by expansion of a polyglutamine (polyQ) tract in the androgen receptor (AR). SBMA is triggered by the interaction between polyQ-AR and its natural ligands, testosterone and dihydrotestosterone (DHT). SBMA is characterized by the loss of lower motor neurons and skeletal muscle fasciculations, weakness, and atrophy. To test the hypothesis that the interaction between polyQ-AR and androgens exerts cell-autonomous toxicity in skeletal muscle, we characterized the process of myogenesis and polyQ-AR expression in DHT-treated satellite cells obtained from SBMA patients and age-matched healthy control subjects. Treatment with androgens increased the size and number of myonuclei in myotubes from control subjects, but not from SBMA patients. Myotubes from SBMA patients had a reduced number of nuclei, suggesting impaired myotube fusion and altered contractile structures. The lack of anabolic effects of androgens on myotubes from SBMA patients was not due to defects in myoblast proliferation, differentiation or apoptosis. DHT treatment of myotubes from SBMA patients increased nuclear accumulation of polyQ-AR and decreased the expression of interleukin-4 (IL-4) when compared to myotubes from control subjects. Following DHT treatment, exposure of myotubes from SBMA patients with IL-4 treatment rescued myonuclear number and size to control levels. This supports the hypothesis that androgens alter the fusion process in SBMA myogenesis. In conclusion, these results provide evidence of an androgen-dependent impairment of myogenesis in SBMA that could contribute to disease pathogenesis.
Website Age:
28 years and 1 months (reg. 1997-05-29).

Matching Content Categories {📚}

  • Science
  • Education
  • Health & Fitness

Content Management System {📝}

What CMS is link.springer.com built with?

Custom-built

No common CMS systems were detected on Link.springer.com, and no known web development framework was identified.

Traffic Estimate {📈}

What is the average monthly size of link.springer.com audience?

🌠 Phenomenal Traffic: 5M - 10M visitors per month


Based on our best estimate, this website will receive around 7,642,828 visitors per month in the current month.

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How Does Link.springer.com Make Money? {💸}

We can't see how the site brings in money.

Not every website is profit-driven; some are created to spread information or serve as an online presence. Websites can be made for many reasons. This could be one of them. Link.springer.com might be plotting its profit, but the way they're doing it isn't detectable yet.

Keywords {🔍}

article, google, scholar, pubmed, cas, muscle, atrophy, androgen, muscular, receptor, spinal, bulbar, sbma, disease, myotubes, skeletal, cells, patients, pennuto, italy, myogenesis, polyglutamine, vergani, expression, model, padova, testosterone, control, number, xlinked, metab, lieberman, mouse, kennedy, cell, privacy, cookies, content, research, androgens, satellite, subjects, treatment, molecular, sobue, clin, endocrinol, university, supplementary, analysis,

Topics {✒️}

month download article/chapter sex-linked recessive trait wild-type androgen receptor muscle stem cells androgen-dependent impairment la spada ar x-linked bulbar ateneo-università di padova spinobulbar muscular atrophy cell-autonomous toxicity full article pdf bulbar muscular atrophy x-linked spinal muscle attenuates disease mutant androgen receptor androgen receptor gene androgen receptor phosphorylation skeletal muscle pathology author information authors phosphorylated akt/akt ratio maria pennuto privacy choices/manage cookies androgen receptor expression skeletal muscle fasciculations human skeletal muscle kennedy disease phenotype molecular mechanisms responsible dht-treated t10 myotubes spinal muscular atrophy kennedy disease knock regulates androgen receptors satellite cells function morgan-hughes ja breedlove sm muscle protein synthesis mammalian muscle growth 50 myotubes/cell line altered contractile structures gianni sorarù dht-treated myoblasts total cell lysates european economic area giovanna cenacchi annarita scaramozza results provide evidence aged symptomatic males illegal performance enhancement harding ae amyotrophic lateral sclerosis allingham-hawkins dj

Schema {🗺️}

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         headline:Androgen-dependent impairment of myogenesis in spinal and bulbar muscular atrophy
         description:Spinal and bulbar muscular atrophy (SBMA) is an inherited neuromuscular disease caused by expansion of a polyglutamine (polyQ) tract in the androgen receptor (AR). SBMA is triggered by the interaction between polyQ-AR and its natural ligands, testosterone and dihydrotestosterone (DHT). SBMA is characterized by the loss of lower motor neurons and skeletal muscle fasciculations, weakness, and atrophy. To test the hypothesis that the interaction between polyQ-AR and androgens exerts cell-autonomous toxicity in skeletal muscle, we characterized the process of myogenesis and polyQ-AR expression in DHT-treated satellite cells obtained from SBMA patients and age-matched healthy control subjects. Treatment with androgens increased the size and number of myonuclei in myotubes from control subjects, but not from SBMA patients. Myotubes from SBMA patients had a reduced number of nuclei, suggesting impaired myotube fusion and altered contractile structures. The lack of anabolic effects of androgens on myotubes from SBMA patients was not due to defects in myoblast proliferation, differentiation or apoptosis. DHT treatment of myotubes from SBMA patients increased nuclear accumulation of polyQ-AR and decreased the expression of interleukin-4 (IL-4) when compared to myotubes from control subjects. Following DHT treatment, exposure of myotubes from SBMA patients with IL-4 treatment rescued myonuclear number and size to control levels. This supports the hypothesis that androgens alter the fusion process in SBMA myogenesis. In conclusion, these results provide evidence of an androgen-dependent impairment of myogenesis in SBMA that could contribute to disease pathogenesis.
         datePublished:2013-05-04T00:00:00Z
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      headline:Androgen-dependent impairment of myogenesis in spinal and bulbar muscular atrophy
      description:Spinal and bulbar muscular atrophy (SBMA) is an inherited neuromuscular disease caused by expansion of a polyglutamine (polyQ) tract in the androgen receptor (AR). SBMA is triggered by the interaction between polyQ-AR and its natural ligands, testosterone and dihydrotestosterone (DHT). SBMA is characterized by the loss of lower motor neurons and skeletal muscle fasciculations, weakness, and atrophy. To test the hypothesis that the interaction between polyQ-AR and androgens exerts cell-autonomous toxicity in skeletal muscle, we characterized the process of myogenesis and polyQ-AR expression in DHT-treated satellite cells obtained from SBMA patients and age-matched healthy control subjects. Treatment with androgens increased the size and number of myonuclei in myotubes from control subjects, but not from SBMA patients. Myotubes from SBMA patients had a reduced number of nuclei, suggesting impaired myotube fusion and altered contractile structures. The lack of anabolic effects of androgens on myotubes from SBMA patients was not due to defects in myoblast proliferation, differentiation or apoptosis. DHT treatment of myotubes from SBMA patients increased nuclear accumulation of polyQ-AR and decreased the expression of interleukin-4 (IL-4) when compared to myotubes from control subjects. Following DHT treatment, exposure of myotubes from SBMA patients with IL-4 treatment rescued myonuclear number and size to control levels. This supports the hypothesis that androgens alter the fusion process in SBMA myogenesis. In conclusion, these results provide evidence of an androgen-dependent impairment of myogenesis in SBMA that could contribute to disease pathogenesis.
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         Androgen receptor
         Dihydrotestosterone
         Myogenesis
         Interleukin-4
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         Neurosciences
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               name:Neurology Unit, Verona Hospital, Verona, Italy
               type:PostalAddress
            type:Organization
      name:Lucia Morandi
      affiliation:
            name:Neurological Institute C. Besta
            address:
               name:Neurological Institute C. Besta, Milan, Italy
               type:PostalAddress
            type:Organization
      name:Elena Pegoraro
      affiliation:
            name:University of Padova
            address:
               name:Department of Neuroscience SNPSRR, University of Padova, Padova, Italy
               type:PostalAddress
            type:Organization
      name:Aaron P. Russell
      affiliation:
            name:Deakin University
            address:
               name:Aaron Russell, Center for Physical Activity and Nutrition Research, School of Exercise and Nutrition Sciences, Deakin University, Burwood, Australia
               type:PostalAddress
            type:Organization
      name:Gianni Sorarù
      affiliation:
            name:University of Padova
            address:
               name:Department of Neuroscience SNPSRR, University of Padova, Padova, Italy
               type:PostalAddress
            type:Organization
      email:[email protected]
      name:Lodovica Vergani
      affiliation:
            name:University of Padova
            address:
               name:Department of Neuroscience SNPSRR, University of Padova, Padova, Italy
               type:PostalAddress
            type:Organization
            name:Centro Biomedico Pietro D’Abano-via Orus 2
            address:
               name:Centro Biomedico Pietro D’Abano-via Orus 2, Padova, Italy
               type:PostalAddress
            type:Organization
      email:[email protected]
PostalAddress:
      name:Department of Neuroscience SNPSRR, University of Padova, Padova, Italy
      name:Department of Neuroscience and Brain Technologies, Istituto Italiano di Tecnologia, Genoa, Italy
      name:Department of Neuroscience SNPSRR, University of Padova, Padova, Italy
      name:Department of Neuroscience SNPSRR, University of Padova, Padova, Italy
      name:Department of Neuroscience SNPSRR, University of Padova, Padova, Italy
      name:IRCCS Istituto Auxologico Italiano, Milan, Italy
      name:Department of Radiological and Histocytopathological Sciences, University of Bologna, Bologna, Italy
      name:Department of Radiological and Histocytopathological Sciences, University of Bologna, Bologna, Italy
      name:Neurology Unit, Verona Hospital, Verona, Italy
      name:Neurological Institute C. Besta, Milan, Italy
      name:Department of Neuroscience SNPSRR, University of Padova, Padova, Italy
      name:Aaron Russell, Center for Physical Activity and Nutrition Research, School of Exercise and Nutrition Sciences, Deakin University, Burwood, Australia
      name:Department of Neuroscience SNPSRR, University of Padova, Padova, Italy
      name:Department of Neuroscience SNPSRR, University of Padova, Padova, Italy
      name:Centro Biomedico Pietro D’Abano-via Orus 2, Padova, Italy
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