Immunohistochemical study of utrophin and dystrophin at the motor end-plate in myasthenia gravis

We studied the densities of utrophin and dystrophin at the motor end-plates of patients with myasthenia gravis (MG) using immunohistochemical analysis. The densities were compared with those found in patients with amyotrophic lateral sclerosis, Lambert-Eaton myasthenic syndrome and normal controls. Utrophin was reduced at the motor end-plates of MG patients, in association with a reduction of alpha-bungarotoxin binding sites. In contrast, the density of dystrophin at the motor end-plate of MG patients was not significantly different from that found in the controls. We conclude that, at the motor end-plate, utrophin may be more closely associated than dystrophin with the acetylcholine receptor, and that it plays a different role.