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NEUROLOGY 2004;62:1778-1782
© 2004 American Academy of Neurology

Expression of protein kinase C isoforms and interleukin-1ß in myofibrillar myopathy

G. Vattemi, MD, P. Tonin, MD, M. Mora, MD, M. Filosto, MD, L. Morandi, MD, C. Savio, BSc, I. Dal Pra, PhD, N. Rizzuto, MD and G. Tomelleri, MD

From the Department of Neurological Sciences and Vision, Section of Clinical Neurology (Drs. Vattemi, Tonin, Filosto, Rizzuto, and Tomelleri, and C. Savio), and Histology and Embryology Unit (Dr. Dal Pra), Clinica Neurologica, Policlinico G.B. Rossi, Verona; and Department of Neuromuscular Diseases, National Institute "C. Besta" (Drs. Mora and Morandi), Milan, Italy.

Address correspondence and reprint requests to Dr. Paola Tonin, Clinica Neurologica, Policlinico G.B. Rossi, Piazzale L.A. Scuro 10, 37134 Verona, Italy; e-mail: paola.tonin{at}azosp.vr.it

Background: The term myofibrillar myopathy refers to a rare and clinically heterogeneous group of muscle disorders. The pathogenesis of this myopathy is not well understood. The morphologic hallmark is myofibrillar destruction with abnormal expression of numerous proteins, most consistently of desmin.

Methods: The authors investigated eight patients with myofibrillar myopathy belonging to four families. They studied the role of different protein kinase C isoforms and of interleukin-1ß, a cytokine that might activate protein kinase C and, in addition, mediate myofibrillar proteolysis.

Results: Immunohistochemical analysis showed the expression of {alpha}, {eta}, and {zeta} isoforms of protein kinase C and of interleukin-1ß in abnormal muscle fibers. Immunoblots confirmed the immunohistochemical data and revealed the absence of protein kinase C {delta} and {epsilon} in muscle fibers from patients and controls.

Conclusions: These data suggest that protein kinase C and interleukin-1ß may play a role in the pathogenesis of myofibrillar myopathy.

Received August 6, 2003. Accepted in final form January 3, 2004.

Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the May 25 issue to find the title link for this article.




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