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NEUROLOGY 1991;41:1977
© 1991 American Academy of Neurology

Progressive depletion of fast alpha-actinin-positive muscle fibers in Duchenne muscular dystrophy

Carlo Minetti, MD, Enzo Ricci, MD and Eduardo Bonilla, MD

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

In normal human muscle, a monoclonal antibody against alpha-actinin recognizes an isoform that is only expressed in a population of fast fibers histochemically identified as type IIb or fast-twitch glycolytic. Immunohistochemical studies of muscle biopsies from patients with Duchenne muscular dystrophy (DMD) showed that the number of alpha-actinin-positive type IIb fibers was essentially normal in preclinical patients. Symptomatic patients between the ages of 3 and 5 years showed depletion of these fibers, which were not seen in patients older than 5 years. ATPase histochemistry showed that a few type IIb fibers were present in muscle from symptomatic DMD patients but lacked the fast isoform of alpha-actinin. The data suggest that type IIb fibers are affected early in DMD.

Address correspondence and reprint requests to Dr. Eduardo Bonilla, Room 5–431, College of Physicians and Surgeons, 630 West 168th Street, New York, NY 10032.

Supported by Center Grant NS 11766 from the National Institute for Neurological Diseases and Stroke, by a grant from the Muscular Dystrophy Association, and by a grant from the Italian Telethon (project # 32).

Received December 18, 1990. Accepted for publication in final form May 13, 1991.




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