Nature Communications<\/a>, scientists at Okayama University describe the molecular pathogenesis of muscular dystrophy-associated cardiomyopathy in mice lacking the fukutin gene (Fktn), the causative gene for Fukuyama muscular dystrophy.<\/p>\nHeart failure is the major cause of death for muscular dystrophy patients; however, little is known for the molecular mechanism of muscular dystrophy-associated cardiomyopathy.<\/p>\n
In this study, a research team spearheaded by Senior Lecturer KATANOSAKA Yuki at Okayama University demonstrate for the first time the cellular and molecular pathomechanisms of muscular dystrophy-associated cardiomyopathy using mouse models of Fukuyama muscular dystrophy with a deficiency for the fukutin gene (Fktn), which encodes a Golgi-based ribitol-phosphate transferase that catalyses the biosynthesis of tandem ribitol-phosphate structure on \u03b1-dystroglycans (DG). As DG and proteins of the dystrophin\u2013glycoprotein complex provide structural support for the sarcolemma in muscle tissue, a loss of membrane fragility was thought to be a cause for cardiac dysfunction in these diseases collectively known as \u03b1-DGpathies. However, their data shows that cardiac dysfunction in muscular dystrophy-associated cardiomyopathy occurs at the cellular cardiomyocyte level.<\/p>\n
Cardiac dysfunction was observed only in later adulthood<\/h3>\n
Although cardiac Fktn elimination markedly reduced \u03b1-DG glycosylation and dystrophin-glycoprotein complex proteins in sarcolemma at all developmental stages, cardiac dysfunction was observed only in later adulthood, suggesting that membrane fragility is not the sole etiology of cardiac dysfunction.<\/p>\n
Younger Fktn-deficient mice show a vulnerability to hemodynamic stress conditions via impaired compensative hypertrophic response of cardiomyocytes. Adult Fktn-deficient mice exhibit altered cardiac morphology and dysfunction, suggesting that FKTN is critical for maintaining contractile function of individual cardiomyocytes.<\/p>\n
In addition, the team show that acute Fktn-elimination causes the disordered Golgi-microtubule network in myocytes. Finally, the team show that treatment with colchicine (an FDA-approved drug for the treatment of familial Mediterranean fever) improved cardiac dysfunction of Fktn-deficient hearts via the recovery of myocyte shortening, which may open a new avenue for therapeutic strategies.<\/p>\n","protected":false},"excerpt":{"rendered":"
Researchers have demonstrated for the first time the cellular and molecular pathomechanisms of muscular dystrophy-associated cardiomyopathy. In a study recently published in Nature Communications, scientists at Okayama University describe the molecular pathogenesis of muscular dystrophy-associated cardiomyopathy in mice lacking the fukutin gene (Fktn), the causative gene for Fukuyama muscular dystrophy. Heart failure is the major […]<\/p>\n","protected":false},"author":4,"featured_media":3746,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"_monsterinsights_skip_tracking":false,"_monsterinsights_sitenote_active":false,"_monsterinsights_sitenote_note":"","_monsterinsights_sitenote_category":0,"footnotes":""},"categories":[10551],"tags":[],"acf":[],"yoast_head":"\n
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