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10.1186/s40478-015-0243-x http://scihub22266oqcxt.onion/10.1186/s40478-015-0243-x C4625612!4625612!26510925     free     free     free Deprecated: Implicit conversion from float 223.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 223.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 223.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 223.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 223.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 223.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 223.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534       Acta+Neuropathol+Commun 2015 ; 3 (ä): ä Nephropedia Template TP {{tp|p=26510925|t=2015. Lack of robust satellite cell activation and muscle regeneration during the progression of Pompe disease |pdf=|usr=}}{{26510925}} gab.com Text Lack of robust satellite cell activation and muscle regeneration during the progression of Pompe disease JO: Acta Neuropathol Commun http://www.kidney.de/pget.php?&tw=1&pmid=26510925 #FOAvip Introduction: Muscle stem cells termed satellite cells are essential for muscle regeneration. A central question in many neuromuscular disorders is why satellite cells are unable to prevent progressive muscle wasting. We have analyzed muscle fiber pathology and the satellite cell response in Pompe disease, a metabolic myopathy caused by acid alpha-glucosidase deficiency and lysosomal glycogen accumulation. Pathology included muscle fiber vacuolization, loss of cross striation, and immune cell infiltration. Results: The total number of Pax7-positive satellite cells in muscle biopsies from infantile, childhood onset and adult patients (with different ages and disease severities) were indistinguishable from controls, indicating that the satellite cell pool is not exhausted in Pompe disease. Pax7/Ki67 double stainings showed low levels of satellite cell proliferation similar to controls, while MyoD and Myogenin stainings showed undetectable satellite cell differentiation. Muscle regenerative activity monitored with expression of embryonic Myosin Heavy Chain was weak in the rapidly progressing classic infantile form and undetectable in the more slowly progressive childhood and adult onset disease including in severely affected patients. Conclusions: These results imply that ongoing muscle wasting in Pompe disease may be explained by insufficient satellite cell activation and muscle regeneration. The preservation of the satellite cell pool may offer a venue for the development of novel treatment strategies directed towards the activation of endogenous satellite cells. Electronic supplementary material: The online version of this article (doi:10.1186/s40478-015-0243-x) contains supplementary material, which is available to authorized users. Twit Text FOAVip Lack of robust satellite cell activation and muscle regeneration during the progression of Pompe disease ????Acta Neuropathol Commun http://www.kidney.de/pget.php?&tw=1&pmid=26510925 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=26510925 #FOAvip English Wikipedia <ref>{{Cite pmid|26510925}}</ref> Lack of robust satellite cell activation and muscle regeneration during the progression of Pompe disease #MMPMID26510925 Schaaf GJ; van Gestel TJ; Brusse E; Verdijk RM; de Coo IF; van Doorn PA; van der Ploeg AT; Pijnappel WP Acta Neuropathol Commun 2015[]; 3 (ä): ä PMID26510925show ga Introduction: Muscle stem cells termed satellite cells are essential for muscle regeneration. A central question in many neuromuscular disorders is why satellite cells are unable to prevent progressive muscle wasting. We have analyzed muscle fiber pathology and the satellite cell response in Pompe disease, a metabolic myopathy caused by acid alpha-glucosidase deficiency and lysosomal glycogen accumulation. Pathology included muscle fiber vacuolization, loss of cross striation, and immune cell infiltration. Results: The total number of Pax7-positive satellite cells in muscle biopsies from infantile, childhood onset and adult patients (with different ages and disease severities) were indistinguishable from controls, indicating that the satellite cell pool is not exhausted in Pompe disease. Pax7/Ki67 double stainings showed low levels of satellite cell proliferation similar to controls, while MyoD and Myogenin stainings showed undetectable satellite cell differentiation. Muscle regenerative activity monitored with expression of embryonic Myosin Heavy Chain was weak in the rapidly progressing classic infantile form and undetectable in the more slowly progressive childhood and adult onset disease including in severely affected patients. Conclusions: These results imply that ongoing muscle wasting in Pompe disease may be explained by insufficient satellite cell activation and muscle regeneration. The preservation of the satellite cell pool may offer a venue for the development of novel treatment strategies directed towards the activation of endogenous satellite cells. Electronic supplementary material: The online version of this article (doi:10.1186/s40478-015-0243-x) contains supplementary material, which is available to authorized users. äLack of robust satellite cell activation and muscle regeneration durin(epmc).pdf DeepDyve Pubget Overpricing