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10.3109/01913123.2013.810684

http://scihub22266oqcxt.onion/10.3109/01913123.2013.810684
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suck abstract from ncbi


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pmid24047347      Ultrastruct+Pathol 2013 ; 37 (5): 304-11
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  • Formation of gelsolin amyloid fibrils in the rough endoplasmic reticulum of skeletal muscle in the gelsolin mouse model of inclusion body myositis Comparative analysis to human sporadic inclusion body myositis #MMPMID24047347
  • Bannykh SI; Balch WE; Page LJ; Shelton GD
  • Ultrastruct Pathol 2013[Oct]; 37 (5): 304-11 PMID24047347show ga
  • Sporadic inclusion body myositis has a significant impact on life of elderly. Despite some similarities to other myopathies with established genetic defects little is known about mechanisms of its development and no effective treatment is available. Therefore there is a need for animal models, which would faithfully reconstitute important aspects of this human disease. We recently expressed mutant form of human gelsolin in mice under control of muscle specific promoter. This induced myopathic changes reminiscent to human inclusion body myositis. In this study we use immunogold labeling to further characterize this model. We are able to demonstrate a presence of gelsolin amyloid deposits within the rough endoplasmic reticulum. We further compare this mouse model to human sporadic inclusion body myositis.
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