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אסיף מאגר המחקר החקלאי
פותח על ידי קלירמאש פתרונות בע"מ -
Prevention of muscle fibrosis and improvement in muscle performance in the mdx mouse by halofuginone
Year:
2008
Source of publication :
Neuromuscular Disorders
Authors :
גנין, אולגה
;
.
חגי, יוסי
;
.
פינס, מרק
;
.
Volume :
18
Co-Authors:
Turgeman, T., Institute of Animal Sciences, The Volcani Center, P.O. Box 6, Bet Dagan, 50250, Israel, Department of Animal Sciences, The Hebrew University of Jerusalem, Rehovot, Israel
Hagai, Y., Department of Animal Sciences, The Hebrew University of Jerusalem, Rehovot, Israel
Huebner, K., Department of Human Anatomy and Cell Science, Faculty of Medicine, University of Manitoba, Canada
Jassal, D.S., Institute of Cardiovascular Sciences, Cardiology Division, Department of Cardiac Sciences, Winnipeg, Man., Canada
Anderson, J.E., Department of Human Anatomy and Cell Science, Faculty of Medicine, University of Manitoba, Canada
Genin, O., Institute of Animal Sciences, The Volcani Center, P.O. Box 6, Bet Dagan, 50250, Israel
Nagler, A., Division of Hematology, Chaim Sheba Medical Center, Tel Hashomer, Israel
Halevy, O., Department of Animal Sciences, The Hebrew University of Jerusalem, Rehovot, Israel
Pines, M., Institute of Animal Sciences, The Volcani Center, P.O. Box 6, Bet Dagan, 50250, Israel
Facilitators :
From page:
857
To page:
868
(
Total pages:
12
)
Abstract:
Fibrosis is a known feature of dystrophic muscles, particularly the diaphragm, in the mdx mouse. In this study we evaluated the effect of halofuginone, a collagen synthesis inhibitor, on collagen synthesis in various muscles of young wild-type (C57/BL/6J) and mdx mice. Halofuginone prevented the age-dependent increase in collagen synthesis in the diaphragms of mdx with no effect on wild-type mice (n = 5 for each time point). This was associated with a decrease in the degenerated areas and number of central nuclei. Halofuginone also inhibited collagen synthesis in cardiac muscle. Moreover, enhanced motor coordination, balance and improved cardiac muscle function were observed implying reduced muscle injury. Halofuginone inhibited Smad3 phosphorylation downstream of TGFβ in the diaphragm and cardiac muscles, in C2 cell line and in primary mouse myoblast cultures representing various muscular dystrophies. We suggest that via its effect on Smad3 phosphorylation, halofuginone inhibits muscle fibrosis and improves cardiac and skeletal muscle functions in mdx mice. © 2008 Elsevier B.V. All rights reserved.
Note:
Related Files :
animal experiment
Animals
animal tissue
Injections, Intraperitoneal
Male
mice
Muscular dystrophy
transforming growth factor beta
עוד תגיות
תוכן קשור
More details
DOI :
10.1016/j.nmd.2008.06.386
Article number:
Affiliations:
Database:
סקופוס
Publication Type:
מאמר
;
.
Language:
אנגלית
Editors' remarks:
ID:
25505
Last updated date:
02/03/2022 17:27
Creation date:
17/04/2018 00:15
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Scientific Publication
Prevention of muscle fibrosis and improvement in muscle performance in the mdx mouse by halofuginone
18
Turgeman, T., Institute of Animal Sciences, The Volcani Center, P.O. Box 6, Bet Dagan, 50250, Israel, Department of Animal Sciences, The Hebrew University of Jerusalem, Rehovot, Israel
Hagai, Y., Department of Animal Sciences, The Hebrew University of Jerusalem, Rehovot, Israel
Huebner, K., Department of Human Anatomy and Cell Science, Faculty of Medicine, University of Manitoba, Canada
Jassal, D.S., Institute of Cardiovascular Sciences, Cardiology Division, Department of Cardiac Sciences, Winnipeg, Man., Canada
Anderson, J.E., Department of Human Anatomy and Cell Science, Faculty of Medicine, University of Manitoba, Canada
Genin, O., Institute of Animal Sciences, The Volcani Center, P.O. Box 6, Bet Dagan, 50250, Israel
Nagler, A., Division of Hematology, Chaim Sheba Medical Center, Tel Hashomer, Israel
Halevy, O., Department of Animal Sciences, The Hebrew University of Jerusalem, Rehovot, Israel
Pines, M., Institute of Animal Sciences, The Volcani Center, P.O. Box 6, Bet Dagan, 50250, Israel
Prevention of muscle fibrosis and improvement in muscle performance in the mdx mouse by halofuginone
Fibrosis is a known feature of dystrophic muscles, particularly the diaphragm, in the mdx mouse. In this study we evaluated the effect of halofuginone, a collagen synthesis inhibitor, on collagen synthesis in various muscles of young wild-type (C57/BL/6J) and mdx mice. Halofuginone prevented the age-dependent increase in collagen synthesis in the diaphragms of mdx with no effect on wild-type mice (n = 5 for each time point). This was associated with a decrease in the degenerated areas and number of central nuclei. Halofuginone also inhibited collagen synthesis in cardiac muscle. Moreover, enhanced motor coordination, balance and improved cardiac muscle function were observed implying reduced muscle injury. Halofuginone inhibited Smad3 phosphorylation downstream of TGFβ in the diaphragm and cardiac muscles, in C2 cell line and in primary mouse myoblast cultures representing various muscular dystrophies. We suggest that via its effect on Smad3 phosphorylation, halofuginone inhibits muscle fibrosis and improves cardiac and skeletal muscle functions in mdx mice. © 2008 Elsevier B.V. All rights reserved.
Scientific Publication
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