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פותח על ידי קלירמאש פתרונות בע"מ -
The involvement of collagen triple helix repeat containing 1 in muscular dystrophies
Year:
2013
Source of publication :
American Journal of Pathology
Authors :
גנין, אולגה
;
.
לביא, עדי
;
.
ספקטור, איתי
;
.
פינס, מרק
;
.
Volume :
182
Co-Authors:
Spector, I., Institute of Animal Sciences, Volcani Center, PO Box 6, Bet Dagan 50250, Israel, Department of Animal Sciences, Hebrew University of Jerusalem, Rehovot, Israel
Zilberstein, Y., Sackler Cellular and Molecular Imaging Center, Tel Aviv University, Tel Aviv, Israel
Lavy, A., Institute of Animal Sciences, Volcani Center, PO Box 6, Bet Dagan 50250, Israel, Department of Animal Sciences, Hebrew University of Jerusalem, Rehovot, Israel
Genin, O., Institute of Animal Sciences, Volcani Center, PO Box 6, Bet Dagan 50250, Israel
Barzilai-Tutsch, H., Department of Animal Sciences, Hebrew University of Jerusalem, Rehovot, Israel
Bodanovsky, A., Department of Animal Sciences, Hebrew University of Jerusalem, Rehovot, Israel
Halevy, O., Department of Animal Sciences, Hebrew University of Jerusalem, Rehovot, Israel
Pines, M., Institute of Animal Sciences, Volcani Center, PO Box 6, Bet Dagan 50250, Israel
Facilitators :
From page:
905
To page:
916
(
Total pages:
12
)
Abstract:
Fibrosis is the main complication of muscular dystrophies. We identified collagen triple helix repeat containing 1 (Cthrc1) in skeletal and cardiac muscles of mice, representing Duchenne and congenital muscle dystrophies (DMD and CMD, respectively), and dysferlinopathy. In all of the mice, Cthrc1 was associated with high collagen type I levels; no Cthrc1 or collagen was observed in muscles of control mice. High levels of Cthrc1 were also observed in biopsy specimens from patients with DMD, in whom they were reversibly correlated with that of β-dystroglycan, whereas collagen type I levels were elevated in all patients with DMD. At the muscle sites where collagen and Cthrc1 were adjacent, collagen fibers appeared smaller, suggesting involvement of Cthrc1 in collagen turnover. Halofuginone, an inhibitor of Smad3 phosphorylation downstream of the transforming growth factor-β signaling, reduced Cthrc1 levels in skeletal and cardiac muscles of mice, representing DMD, CMD, and dysferlinopathy. The myofibroblasts infiltrating the dystrophic muscles of the murine models of DMD, CMD, and dysferlinopathy were the source of Cthrc1. Transforming growth factor-β did not affect Cthrc1 levels in the mdx fibroblasts but decreased them in the control fibroblasts, in association with increased migration of mdx fibroblasts and dystrophic muscle invasion by myofibroblasts. To our knowledge, this is the first demonstration of Cthrc1 as a marker of the severity of the disease progression in the dystrophic muscles, and as a possible target for therapy. Copyright © 2013 American Society for Investigative Pathology.
Note:
Related Files :
animal experiment
animal model
Animals
Extracellular Matrix Proteins
gene expression
mice
Muscular dystrophy
transforming growth factor beta
עוד תגיות
תוכן קשור
More details
DOI :
10.1016/j.ajpath.2012.11.004
Article number:
0
Affiliations:
Database:
סקופוס
Publication Type:
מאמר
;
.
Language:
אנגלית
Editors' remarks:
ID:
31759
Last updated date:
02/03/2022 17:27
Creation date:
17/04/2018 01:05
You may also be interested in
Scientific Publication
The involvement of collagen triple helix repeat containing 1 in muscular dystrophies
182
Spector, I., Institute of Animal Sciences, Volcani Center, PO Box 6, Bet Dagan 50250, Israel, Department of Animal Sciences, Hebrew University of Jerusalem, Rehovot, Israel
Zilberstein, Y., Sackler Cellular and Molecular Imaging Center, Tel Aviv University, Tel Aviv, Israel
Lavy, A., Institute of Animal Sciences, Volcani Center, PO Box 6, Bet Dagan 50250, Israel, Department of Animal Sciences, Hebrew University of Jerusalem, Rehovot, Israel
Genin, O., Institute of Animal Sciences, Volcani Center, PO Box 6, Bet Dagan 50250, Israel
Barzilai-Tutsch, H., Department of Animal Sciences, Hebrew University of Jerusalem, Rehovot, Israel
Bodanovsky, A., Department of Animal Sciences, Hebrew University of Jerusalem, Rehovot, Israel
Halevy, O., Department of Animal Sciences, Hebrew University of Jerusalem, Rehovot, Israel
Pines, M., Institute of Animal Sciences, Volcani Center, PO Box 6, Bet Dagan 50250, Israel
The involvement of collagen triple helix repeat containing 1 in muscular dystrophies
Fibrosis is the main complication of muscular dystrophies. We identified collagen triple helix repeat containing 1 (Cthrc1) in skeletal and cardiac muscles of mice, representing Duchenne and congenital muscle dystrophies (DMD and CMD, respectively), and dysferlinopathy. In all of the mice, Cthrc1 was associated with high collagen type I levels; no Cthrc1 or collagen was observed in muscles of control mice. High levels of Cthrc1 were also observed in biopsy specimens from patients with DMD, in whom they were reversibly correlated with that of β-dystroglycan, whereas collagen type I levels were elevated in all patients with DMD. At the muscle sites where collagen and Cthrc1 were adjacent, collagen fibers appeared smaller, suggesting involvement of Cthrc1 in collagen turnover. Halofuginone, an inhibitor of Smad3 phosphorylation downstream of the transforming growth factor-β signaling, reduced Cthrc1 levels in skeletal and cardiac muscles of mice, representing DMD, CMD, and dysferlinopathy. The myofibroblasts infiltrating the dystrophic muscles of the murine models of DMD, CMD, and dysferlinopathy were the source of Cthrc1. Transforming growth factor-β did not affect Cthrc1 levels in the mdx fibroblasts but decreased them in the control fibroblasts, in association with increased migration of mdx fibroblasts and dystrophic muscle invasion by myofibroblasts. To our knowledge, this is the first demonstration of Cthrc1 as a marker of the severity of the disease progression in the dystrophic muscles, and as a possible target for therapy. Copyright © 2013 American Society for Investigative Pathology.
Scientific Publication
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