{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T21:39:18Z","timestamp":1790890758791,"version":"4.1.0"},"reference-count":11,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2001,5,15]],"date-time":"2001-05-15T00:00:00Z","timestamp":989884800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2001,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Electron microscopy study of muscle biopsies from patients with autosomal\u2010dominant Emery\u2010Dreifuss muscular dystrophy revealed nuclear alterations in about 10% of the preserved muscle fibers. The major findings consisted of peripheral heterochromatin loss or detachment from the nuclear envelope, and of interchromatin texture alterations. These abnormalities are similar to those reported in an animal model of the disease and to those found in the X\u2010linked form of Emery\u2010Dreifuss muscular dystrophy. These results suggest that an abnormal ultrastructural arrangement of the nuclear periphery is a common feature in the known forms of Emery\u2010Dreifuss muscular dystrophy, and that several proteins of the nuclear scaffold are necessary in muscle cells to maintain the nuclear structural\/functional integrity and a normal muscle cell metabolism. \u00a9 2001 John Wiley &amp; Sons, Inc. 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