Three muscular dystrophies: loss of cytoskeleton-extracellular matrix linkage
Article Abstract:
Pathogenesis of the three human muscular dystrophies, Duchenne muscular dystrophy (DMD), severe childhood autosomal recessive muscular dystrophy (SCARMD) and congenital muscular dystrophy (CMD), involves the dystrophin-glycoprotein complex. Linkage between extracellular matrix and cytoskeleton in skeletal muscles occurs through this complex. All three forms of dystrophy exhibit biochemical deficiencies of elements of the component, dystrophin gene mutation in DMD, merosin deficiency in CMD and adhalin deficiency in SCARMD. Reestablishment of the attachment between extracellular matrix and cytoskeleton is essential for the effective gene therapy of the three muscular dystrophy forms.
Publication Name: Cell
Subject: Biological sciences
ISSN: 0092-8674
Year: 1995
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Stress pathways and heart failure
Article Abstract:
Stress pathways and heart failure are discussed, and the dilated cardiomyopathy puzzle is linked to such pathways in this review article. Puzzle pieces include distinct biomechanical stress pathways, myocyte survival, apoptosis pathways, and calcium cycling defects. It may be possible in time to reverse the dilated cardiomypathy process by cutting down on wall stress at the molecular level. Recent work describing rare monogenic forms of human dilated cardiomyopathy and gene-targeted mouse models of muscle-specific mutations has made possible identification of genetic pathways that can lead to dilated cardiomyopathy, which often has a genetic component.
Publication Name: Cell
Subject: Biological sciences
ISSN: 0092-8674
Year: 1999
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