Analysis of dystrophin in muscular diseases by two-dimensional gel electrophoresis using agarose gels in the first dimension

K. Hamano, T. Takeya, N. Iwasaki, N. Okoshi, T. Fukubayashi, M. Kirinoki, Y. Yao, T. Hirabayashi, H. Takita

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Abstract

We analyzed dystrophin in cases of normal control, Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD) and infectious muscular disease using two-dimensional gel electrophoresis and immunoblotting with 3 monoclonal dystrophin antibodies : Dys L a mid-rod-domain antibody ; Dys 2, a C-terminal-domain antibody ; and Dys 3, an N-terminal-domain antibody. In cases of normal control, a clearly separated doublet of bands was observed for Dys 1 and 3 at molecular weights 400 and 420 kDa. The isoelectric point was between pH ∼ 5.7-∼ 5.9, similar to that for the myosin heavy chain. In one DMD case, a single faint band was observed for Dys 2. BMD presented a single-band pattern for each antibody. Infectious diseases cases showed 3- to 5-band patterns for Dys 1 and single or no bands for Dys 2 and 3. The pI of the Dys 1 band was almost identical. These results suggest coexistence of normal dystrophin and its proteolytic products, both containing triple helical segment, and show that two-dimensional gel electrophoresis may be applicable in the analysis of dystrophin in muscular disease.

Original languageEnglish
Pages (from-to)102-107
Number of pages6
JournalActa Neurologica Belgica
Volume96
Issue number2
Publication statusPublished - 1996
Externally publishedYes

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Keywords

  • Becker muscular dystrophy
  • Duchenne muscular dystrophy
  • Gel electrophoresis
  • Infectious muscular disease
  • Two-dimensional

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Hamano, K., Takeya, T., Iwasaki, N., Okoshi, N., Fukubayashi, T., Kirinoki, M., Yao, Y., Hirabayashi, T., & Takita, H. (1996). Analysis of dystrophin in muscular diseases by two-dimensional gel electrophoresis using agarose gels in the first dimension. Acta Neurologica Belgica, 96(2), 102-107.