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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Ultrastruct+Pathol
2013 ; 37
(5
): 304-11
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
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English Wikipedia
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
; Kelly JW
; Page LJ
; Shelton GD
Ultrastruct Pathol
2013[Oct]; 37
(5
): 304-11
PMID24047347
show ga
Sporadic inclusion body myositis has a significant impact on the life of the
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 that can
faithfully reconstitute important aspects of this human disease. The authors
recently expressed a mutant form of human gelsolin in mice under the control of a
muscle-specific promoter. This induced myopathic changes reminiscent of human
inclusion body myositis. In this study, immunogold labeling is used to further
characterize this model. The study demonstrates a presence of gelsolin amyloid
deposits within the rough endoplasmic reticulum. It further compares this mouse
model to human sporadic inclusion body myositis.