Regulation of the calpain and ubiquitin-proteasome systems in a canine model of muscular dystrophy.

Regulation of the calpain and ubiquitin-proteasome systems in a canine model of muscular dystrophy.
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DOI:
10.1002/mus.22125
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发表时间:
2011-10
期刊:
影响因子:
3.4
通讯作者:
Willis, Monte
Willis, Monte
中科院分区:
医学3区
文献类型:
--
作者:
Wadosky, Kristine M.;Bs, Luge Li;Rodriguez, Jessica E.;Min, Jin-na;Bogan, Dan;Gonzalez, Jason;Patterson, Cam;Kornegay, Joe N.;Willis, Monte

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Previous studies have tested the hypothesis that calpain and/or proteasome inhibition is beneficial in Duchenne Muscular Dystrophy, based largely on evidence that calpain and proteasome activities are enhanced in the mdx mouse. mRNA expression of ubiquitin proteasome and calpain system components were determined using RT-PCR in skeletal muscle and heart in the golden retriever muscular dystrophy model. Similarly, calpain 1/2 and proteasome activities were determined using fluorometric activity assays. We found that less than half of the muscles tested had increases in proteasome activity, and only one-half had increased calpain activity. Additionally, transcriptional regulation of the ubiquitin proteasome system was most pronounced in the heart, where numerous components were significantly decreased. This study illustrates the diversity of expression and activities of the ubiquitin proteasome and calpain systems, which may lead to unexpected consequences in response to pharmacologic inhibition.
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