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Immunohistological analyses of neutral glycosphingolipids and gangliosides in normal mouse skeletal muscle and in mice with neuromuscular diseases

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Abstract

The expression of neutral glycosphingolipids (GSLs) and gangliosides was investigated in cryosections of normal mouse skeletal muscle and in muscle of mice with neuromuscular diseases using indirect immunofluorescence microscopy. Transversal and longitudinal sections were immunostained with specific polyclonal antibodies against lactosylceramide, lacto-N-neotetraosylceramide, globoside, GM3(Neu5Ac), GM3(Neu5Gc) and GM1(Neu5Ac) as well as monoclonal anti-Forssman GSL antibody. In normal CBA/J mouse muscle (control) the main immunohistochemically detected ganglioside was GM3(Neu5Ac) followed by moderately expressed GM3(Neu5Gc) and GM1. The neutral GSLs lactosylceramide and globoside were stained with almost identical, high fluorescence intensity. Low amounts of lacto-N-neotetraosylceramide and trace quantities of Forssman GSL were immunostained. All GSLs were detected in the sarcolemma, but also in considerable amounts at the intracellular level. Mice with neuromuscular diseases were the A2G-adr mouse mutant (a model for human recessive myotonia of Becker type), the BL6-wr mutant (a model for motor neuron disease) and the BL10-mdx mouse mutant (a model for human Duchenne muscular dystrophy). No changes in GSL expression were found in the A2G-adr mouse, while muscle of the BL6-wr mouse showed increased intensity of immunofluorescence in stainings with anti-lactosylceramide and anti-GM3(Neu5Ac) antibodies. Muscle of BL10-mdx mice showed the most prominent changes in GSL expression with reduced fluorescence intensity for all antibodies. Major differences were not observed in the intensities of GSLs, but there were significant differences in the patterns of distribution on plasma membrane and at the subcellular level. The exact nature and pathogenesis of these changes should be elucidated since such investigations could furnish advances in understanding the functional role of neutral GSLs and gangliosides in normal as well as in diseased muscle.

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Authors and Affiliations

  1. Department of Chemistry and Biochemistry, School of Medicine, University of Zagreb, Šalata 3b, 41000, Zagreb, Croatia

    Melita Čačić & Ksenija Šoštarić

  2. Developmental Biology Unit, University of Bielefeld, 33501, Bielefeld, Germany

    Sabine Weber-Schürholz

  3. Institute of Cell Culture Technology, University of Bielefeld, Postfach 100131, 33501, Bielefeld, Germany

    Johannes Müthing

Authors
  1. Melita Čačić
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  2. Ksenija Šoštarić
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  3. Sabine Weber-Schürholz
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  4. Johannes Müthing
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Additional information

Abbreviations: BSA, bovine serum albumin; DAPI, 4′, 6-diamidine-2-phenylindole-dihydrochloride; DTAF, dichlorotriazinylamino-fluorescein; GSL(s), glycosphingolipid(s); Neu5Ac,N-acetylneuraminic acid; Neu5Gc,N-glycolylneuraminic acid [53]; PBS, phosphate buffered saline. The designation of the following glycosphingolipids follows the IUPAC-IUB recommendations [54] and the nomenclature of Svennerholm [55]. Lactosylceramide or LacCer, Galβ1-4Glcβ1-1Cer; gangliotriaosylceramide or GgOse3Cer, GalNAcβ1-4Galβ1-4Glcβ1-1Cer; globotriaosylceramide or GbOse3Cer, Galα1-4Galβ1-4Glcβ1-1Cer; gangliotetraosylceramide or GgOse3Cer, Galβ1-3GalNAcβ1-4Galβ1-4Glcβ1-1Cer; globotetraosylceramide or GbOse4Cer, GalNAcβ1-3Galα1-4Galβ1-4Glcβ1-1Cer; lacto-N-neotetraosylceramide or nLcOse4Cer, Galβ1-4GlcNAcβ1-3Galβ1-4Glcβ1-1Cer; Forssman GSL or GbOse5Cer, GalNAcα1-3GalNAcβ1-3GAlα1-4Galβ1-4Glcβ1-1Cer; GM3, II3Neu5Ac-LacCer; GM1, II3Neu5Ac-GgOse4Cer.

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Čačić, M., Šoštarić, K., Weber-Schürholz, S. et al. Immunohistological analyses of neutral glycosphingolipids and gangliosides in normal mouse skeletal muscle and in mice with neuromuscular diseases. Glycoconjugate J 12, 721–728 (1995). https://doi.org/10.1007/BF00731270

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  • DOI: https://doi.org/10.1007/BF00731270

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