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Abnormalities in the expression of ß-spectrin in Duchenne muscular dystrophy

Department of Neurology (Drs. Minetti, Tanji, Gasparo Rippa, and Bonilla), College of Physicians and Surgeons of Columbia University, New York, NY, and the Department of Pediatrics (Drs. Minetti, Morreale, and Cordone), Instituto G. Gaslini, Genova, Italy.

We studied (ß-spectrin using an immunologic probe in muscle samples from patients with Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), other disease controls, and normal controls. By immunohistochemistry in DMD samples, ß-spectrin showed reduced or interrupted staining of the entire cell surface or only patches of bright staining at the cell periphery. There were also alterations of ß-spectrin immunostain in fibers that were not degenerating or regenerating. By immunoblotting, the amount of ß-spectrin in muscle was reduced and varied from 52% to 78% of the normal controls. We found normal values of ß-spectrin in BMD and disease controls. These observations indicate that the expression of ß-spectrin in DMD is abnormal and that ß-spectrin immunolabeling is not a good marker for monitoring membrane integrity in DMD muscle.

Address correspondence and reprint requests to Dr. Eduardo Bonilla, Room 5-431, College of Physicians and Surgeons, New York, NY 10032.

Supported by Center Grant NS 11766 from the National Institute of Neurological Diseases and Stroke, by a grant from the Muscular Dystrophy Association (E.B.), and by a grant from the Italian Muscular Dystrophy Telethon, project 31 (C.M.).

Received August 18, 1993. Accepted for publication in final form December 1, 1993.

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