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

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

Research output: Contribution to journalArticle

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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Spectrin
Duchenne Muscular Dystrophy
Muscles
Staining and Labeling
Muscular Diseases
Immunoblotting
Reference Values
Immunohistochemistry
Membranes
Cells

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.

Abnormalities in the expression of β–spectrin in duchenne muscular dystrophy. / Minetti, C.; Tanji, K.; Rippa, P. Gasparo; Morreale, G.; Cordone, C.; Bonilla, Eduardo.

In: Neurology, Vol. 44, No. 6, 1994, p. 1149-1153.

Research output: Contribution to journalArticle

Minetti, C, Tanji, K, Rippa, PG, Morreale, G, Cordone, C & Bonilla, E 1994, 'Abnormalities in the expression of β–spectrin in duchenne muscular dystrophy', Neurology, vol. 44, no. 6, pp. 1149-1153.
Minetti C, Tanji K, Rippa PG, Morreale G, Cordone C, Bonilla E. Abnormalities in the expression of β–spectrin in duchenne muscular dystrophy. Neurology. 1994;44(6):1149-1153.
Minetti, C. ; Tanji, K. ; Rippa, P. Gasparo ; Morreale, G. ; Cordone, C. ; Bonilla, Eduardo. / Abnormalities in the expression of β–spectrin in duchenne muscular dystrophy. In: Neurology. 1994 ; Vol. 44, No. 6. pp. 1149-1153.
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