Focusing on fibrosis: halofuginone-induced functional improvement in the mdx mouse model of Duchenne muscular dystrophy.
نویسنده
چکیده
Corresponding Author: Linda K McLoon, Ph.D. Department of Ophthalmology University Of Minnesota Room 374 LRB, 2001 6 Street SE Minneapolis, MN 55455 Tel: 612-626-0777 Fax: 612-626-0781 Email: [email protected] Running title: Focusing on Fibrosis Submitted as an Editorial Focus to American Journal of Physiology: Heart and Circulatory Physiology Articles in PresS. Am J Physiol Heart Circ Physiol (February 29, 2008). doi:10.1152/ajpheart.00176.2008
منابع مشابه
Functional resolution of fibrosis in mdx mouse dystrophic heart and skeletal muscle by halofuginone.
The effect of halofuginone (Halo) on established fibrosis in older mdx dystrophic muscle was investigated. Mice (8 to 9 mo) treated with Halo (or saline in controls) for 5, 10, or 12 wk were assessed weekly for grip strength and voluntary running. Echocardiography was performed at 0, 5, and 10 wk. Respiratory function and exercise-induced muscle damage were tested. Heart, quadriceps, diaphragm,...
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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 ...
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Duchenne muscular dystrophy (DMD) is caused by dystrophin deficiency resulting in progressive muscle weakness and fibrotic scarring. Muscle fibrosis impairs blood flow, hampering muscle repair and regeneration. Irrespective of the success of gene restoration, functional improvement is limited without reducing fibrosis. The levels of miR-29c, a known regulator of collagen, are reduced in DMD. Ou...
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Various therapeutic approaches have been studied for the treatment of Duchenne muscular dystrophy (DMD), but none of these approaches have led to significant long-term effects in patients. One reason for this observed inefficacy may be the use of inappropriate animal models for the testing of therapeutic agents. The mdx mouse is the most widely used murine model of DMD, yet it does not model th...
متن کاملKlotho gene silencing promotes pathology in the mdx mouse model of Duchenne muscular dystrophy.
Duchenne muscular dystrophy (DMD) is a lethal muscle disease involving progressive loss of muscle regenerative capacity and increased fibrosis. We tested whether epigenetic silencing of the klotho gene occurs in the mdx mouse model of DMD and whether klotho silencing is an important feature of the disease. Our findings show that klotho undergoes muscle-specific silencing at the acute onset of m...
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عنوان ژورنال:
- American journal of physiology. Heart and circulatory physiology
دوره 294 4 شماره
صفحات -
تاریخ انتشار 2008