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NEUROLOGY 1992;42:1751
© 1992 American Academy of Neurology

Immunologic study of vinculin in Duchenne muscular dystrophy

Carlo Minetti, MD, Kurenai Tanji, MD and Eduardo Bonilla, MD

Department of Neurology, College of Physicians and Surgeons of Columbia University, New York, NY (Drs. Minetti, Tanji, and Bonilla)
Department of Pediatrics, Istituto G. Gaslini, Geneva, Italy (Dr. Minetti).

Using immunologic techniques, we studied vinculin, a cytoskeletal protein associated with the membrane-skeleton of the muscle fiber. We examined muscle biopsies from five patients with Duchenne muscular dystrophy (DMD), two with Becker's muscular dystrophy (BMD), three normal human muscle samples, and four biopsies from disease control patients. All DMD patients showed patchy and low-intensity immunostain at the sarcolemma of most fibers and, by immunoblot analysis, the content of vinculin was 42 to 61% of control values. There was no significant vinculin deficiency in samples from patients with BMD and other disease controls. The data suggest that vinculin content is reduced only in muscle where dystrophin is absent or sparse.

Supported by Center Grant NS 11766 from the National Institute for 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 32 (CM.).

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

Received November 7, 1991. Accepted for publication in final form February 13, 1992.




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