Abnormalities in the expression of β–spectrin in duchenne muscular dystrophy

C. Minetti, K. Tanji, P. Gasparo Rippa, G. Morreale, C. Cordone, Eduardo Bonilla

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Abstract

We studied (β-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, β-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 β-spectrin immunostain in fibers that were not degenerating or regenerating. By immunoblotting, the amount of β-spectrin in muscle was reduced and varied from 52% to 78% of the normal controls. We found normal values of β-spectrin in BMD and disease controls. These observations indicate that the expression of β-spectrin in DMD is abnormal and that β-spectrin immunolabeling is not a good marker for monitoring membrane integrity in DMD muscle.

Original languageEnglish
Pages (from-to)1149-1153
Number of pages5
JournalNeurology
Volume44
Issue number6
Publication statusPublished - 1994

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ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology
  • Neuroscience(all)

Cite this

Minetti, C., Tanji, K., Rippa, P. G., Morreale, G., Cordone, C., & Bonilla, E. (1994). Abnormalities in the expression of β–spectrin in duchenne muscular dystrophy. Neurology, 44(6), 1149-1153.