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(Circulation. 2004;110:3290-3292.)
© 2004 American Heart Association, Inc.

Editorial

Regenerating More Than Muscle in Muscular Dystrophy

Ahlke Heydemann, PhD; Elizabeth M. McNally, MD, PhD

From the Department of Medicine (A.H., E.M.M.) and Department of Human Genetics (E.M.M.), The University of Chicago, Ill.

Correspondence to E.M. McNally, Section of Cardiology, Department of Medicine, 5841 S Maryland Ave, MC6088, The University of Chicago, Chicago, IL 60637. E-mail emcnally@medicine.bsd.uchicago.edu

Key Words: Editorials • dystrophin • muscular dystrophies • angiogenesis

An extract of the first 250 words of the full text is provided, because this article has no abstract.

Duchenne muscular dystrophy (DMD) is a progressive muscle-wasting disorder of skeletal and cardiac muscle. Mutations in the dystrophin gene produce DMD, but much is still unknown about the pathophysiology of DMD. Dystrophic muscle is defined by replacement of normal myofibers by connective tissue and adipocytes. Normal skeletal muscle is highly regenerative and, in the face of continued degeneration, muscle regeneration in DMD is robust, although insufficient to match the pace of degeneration. The dystrophin protein is a subsarcolemmal, spectrin repeat–containing protein of skeletal muscle myofibers and cardiomyocytes. Dystrophin associates tightly with a constellation of transmembrane proteins forming the dystrophin-glycoprotein complex (DGC). Extracellularly, the DGC binds to laminin 2, and intracellularly, the DGC binds to cytoplasmic -actin, a myocyte-specific form of filamin and neuronal nitric oxide synthase (nNOS). Positioned at the plasma membrane, the DGC is a mechanosignaling complex that connects the extracellular matrix with the cytoskeleton, and dystrophin is central to this role.¹ When dystrophin is absent, as is the case in DMD, the entire DGC is destabilized. The dissolution of the DGC produces plasma membrane instability associated with increased intracellular calcium and myofiber degeneration.

See p 3341

The ongoing and widespread muscle degeneration in DMD produces a number of systemic responses. In the present issue of Circulation, Straino and colleagues² now show that arteriogenesis is enhanced in mdx mice, a mouse model of DMD. Under ischemic conditions, an increase in hindlimb perfusion was seen in mdx as compared with control mice. As an explanation of increased perfusion, muscle . . . [Full Text of this Article]

Related Article:

Enhanced Arteriogenesis and Wound Repair in Dystrophin-Deficient mdx Mice

Stefania Straino, Antonia Germani, Anna Di Carlo, Daniele Porcelli, Roberta De Mori, Antonella Mangoni, Monica Napolitano, Fabio Martelli, Paolo Biglioli, and Maurizio C. Capogrossi
Circulation 2004 110: 3341-3348. [Abstract] [Full Text]

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