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פותח על ידי קלירמאש פתרונות בע"מ -
Inhibition of muscle fibrosis results in increases in both utrophin levels and the number of revertant myofibers in Duchenne muscular dystrophy
Year:
2015
Source of publication :
Oncotarget
Authors :
גנין, אולגה
;
.
לוי, אשרת
;
.
פינס, מרק
;
.
Volume :
6
Co-Authors:
Levi, O., Institute of Animal Sciences, Volcani Center, Bet Dagan, Israel
Genin, O., Institute of Animal Sciences, Volcani Center, Bet Dagan, Israel
Angelini, C., Department of Neurosciences, University of Padova, IRCCS S. Camillo, Lido, Venice, Italy
Halevy, O., Department of Animal Sciences, The Hebrew University of Jerusalem, Rehovot, Israel
Pines, M., Institute of Animal Sciences, Volcani Center, Bet Dagan, Israel
Facilitators :
From page:
23249
To page:
23260
(
Total pages:
12
)
Abstract:
Duchenne Muscular Dystrophy is characterized by: near absence of dystrophin in skeletal muscles; low percentage of revertant myofibers; up-regulation of utrophin synthesis; and a high degree of muscle fibrosis. In patient quadriceps femoris biopsies (n = 6, ages between 3-9 years) an inverse correlation was observed between the levels of collagen type I - representing fibrosis - and the levels of utrophin. This correlation was independent of the patient's age and was observed in the entire muscle biopsy sections. In the mdx mice diaphragm (n = 6/group), inhibition of fibrosis by halofuginone resulted in increases in the levels of utrophin. The utrophin/ fibrosis relationships were not limited to collagen type I, but also applied to other constituents of the fibrosis machinery. The inverse correlation was found also in old mdx mice with established fibrosis. In addition, inhibition of collagen type I levels was associated with increases in the numbers of revertant myofibers, both as single myofibers and in clusters in the diaphragm and the gastrocnemius. In summary, our results demonstrate an inverse correlation between the level of muscle fibrosis and the level of utrophin and that of the number of revertant myofibers. These findings may reveal common links between the fibrotic and utrophinsynthesis pathways and offer new insights into the regulation of utrophin synthesis.
Note:
Related Files :
Animal
Animals
animal tissue
Male
metabolism
mice
Muscular dystrophy
Pathology
quinazolinone derivative
עוד תגיות
תוכן קשור
More details
DOI :
10.18632/oncotarget.4021
Article number:
0
Affiliations:
Database:
סקופוס
Publication Type:
מאמר
;
.
Language:
אנגלית
Editors' remarks:
ID:
26736
Last updated date:
02/03/2022 17:27
Creation date:
17/04/2018 00:25
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Scientific Publication
Inhibition of muscle fibrosis results in increases in both utrophin levels and the number of revertant myofibers in Duchenne muscular dystrophy
6
Levi, O., Institute of Animal Sciences, Volcani Center, Bet Dagan, Israel
Genin, O., Institute of Animal Sciences, Volcani Center, Bet Dagan, Israel
Angelini, C., Department of Neurosciences, University of Padova, IRCCS S. Camillo, Lido, Venice, Italy
Halevy, O., Department of Animal Sciences, The Hebrew University of Jerusalem, Rehovot, Israel
Pines, M., Institute of Animal Sciences, Volcani Center, Bet Dagan, Israel
Inhibition of muscle fibrosis results in increases in both utrophin levels and the number of revertant myofibers in Duchenne muscular dystrophy
Duchenne Muscular Dystrophy is characterized by: near absence of dystrophin in skeletal muscles; low percentage of revertant myofibers; up-regulation of utrophin synthesis; and a high degree of muscle fibrosis. In patient quadriceps femoris biopsies (n = 6, ages between 3-9 years) an inverse correlation was observed between the levels of collagen type I - representing fibrosis - and the levels of utrophin. This correlation was independent of the patient's age and was observed in the entire muscle biopsy sections. In the mdx mice diaphragm (n = 6/group), inhibition of fibrosis by halofuginone resulted in increases in the levels of utrophin. The utrophin/ fibrosis relationships were not limited to collagen type I, but also applied to other constituents of the fibrosis machinery. The inverse correlation was found also in old mdx mice with established fibrosis. In addition, inhibition of collagen type I levels was associated with increases in the numbers of revertant myofibers, both as single myofibers and in clusters in the diaphragm and the gastrocnemius. In summary, our results demonstrate an inverse correlation between the level of muscle fibrosis and the level of utrophin and that of the number of revertant myofibers. These findings may reveal common links between the fibrotic and utrophinsynthesis pathways and offer new insights into the regulation of utrophin synthesis.
Scientific Publication
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