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Freeze-fracture studies of muscle plasma membrane in Fukuyama-type congenital muscular dystrophy

From the Division of Neurology, Department of Medicine (Drs. Wakayama and Shibuya), Showa University Fujigaoka Hospital, Yokohama; and the Division of Pediatric Neurology (Dr. Kumagai), Central Hospital, Aichi Prefectural Colony, Kasugai, Japan.

We used freeze-fracture to study muscle plasma membrane in six patients with Fukuyama-type congenital muscular dystrophy and six control children. In the patients, there was significantly fewer intramembranous particles (IMPs) and orthogonal arrays in the P face, with less conspicuous depletion of IMPs in E face. However, the density of caveolae was not affected.

Address correspondence and reprint requests to Dr. Wakayama, Division of Neurology, Department of Medicine, Showa University Fujigaoka Hospital, 1–30 Fujigaoka, Midori-ku, Yokohama, 227, Japan.

Supported by the grant from the National Center for Nervous, Mental and Muscular Disorders of the Ministry of Health and Welfare (No. 84-08-35), Japan.

Accepted for pubhation March 7, 1985.