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Immunoblot analysis of sarcoplasmic calcium binding proteins in Duchenne muscular dystrophy

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Abstract

The Western blotting technique was used to detect parvalbumin and S-100 protein in muscles from 10 Duchenne muscular dystrophy (DD) patients, 13 patients with other muscle diseases and 5 age-matched healthy subjects. DD muscles were found to contain decreased amounts of parvalbumin and the S-100 protein. The parvalbumin level did not relate to the age of the patients and the stage of the disease. The S-100 protein decreased progressively with the age of the patients. In a very advanced DD case the S-100 protein was present in trace amounts. In other primary myopathies, including Becker dystrophy, and neurogenic muscular atrophy both parvalbumin and S-100 protein levels were similar to that observed in healthy subjects. The decrease in the amount of both calcium binding proteins may contribute to the elevation of free intracellular Ca2+ level in the sarcoplasm of dystrophic muscle and would result in abnormalities in processes regulated by these proteins. The mechanism(s) responsible for the decrease of parvalbumin and S-100 protein in DD muscles are discussed.

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Authors and Affiliations

  1. Department of Neurology, Medical Academy, 1a Banacha Street, 02-097, Warsaw, Poland

    Irena Niebrój-Dobosz & Miroslawa Łukasiuk

  2. The Neuromuscular Unit, Medical Research Center, Polish Academy of Sciences, 1a Banacha Street, 02-097, Warsaw, Poland

    I. Niebrój-Dobosz

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  1. Irena Niebrój-Dobosz
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  2. Miroslawa Łukasiuk
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  3. I. Niebrój-Dobosz
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Niebrój-Dobosz, I., Łukasiuk, M. & Niebrój-Dobosz, I. Immunoblot analysis of sarcoplasmic calcium binding proteins in Duchenne muscular dystrophy. J Neurol 242, 82–86 (1995). https://doi.org/10.1007/BF00887821

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  • DOI: https://doi.org/10.1007/BF00887821

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