Focusing on fibrosis: halofuginone-induced functional improvement in the mdx mouse model of Duchenne muscular dystrophy.

نویسنده

  • Linda K McLoon
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

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Functional resolution of fibrosis in mdx mouse dystrophic heart and skeletal muscle by halofuginone.

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Inhibition of muscle fibrosis results in increases in both utrophin levels and the number of revertant myofibers in Duchenne muscular dystrophy

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MicroRNA-29 overexpression by adeno-associated virus suppresses fibrosis and restores muscle function in combination with micro-dystrophin.

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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Skeletal Muscle Fibrosis in the mdx/utrn+/- Mouse Validates Its Suitability as a Murine Model of Duchenne Muscular Dystrophy

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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