Evaluation of sarcoglycans, vinculin-talin-integrin system and filamin2 in α- and γ-sarcoglycanopathy: An immunohistochemical study

  • Authors:
    • Giuseppe Anastasi
    • Giuseppina Cutroneo
    • Fabio Trimarchi
    • Giuseppe Santoro
    • Daniele Bruschetta
    • Placido Bramanti
    • Antonina Pisani
    • Angelo Favaloro
  • View Affiliations

  • Published online on: December 1, 2004     https://doi.org/10.3892/ijmm.14.6.989
  • Pages: 989-999
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Abstract

The sarcoglycan subcomplex (SGC) is a well-known system of interaction between extracellular matrix and sarcolemma-associated cytoskeleton in skeletal and cardiac muscle. The SGC is included in the DGC made up of sarcoplasmic subcomplex and a dystroglycan subcomplex. Recent developments in molecular genetics have demonstrated that the mutation of each single sarcoglycan gene, causes a series of recessive autosomal muscular dystrophies, dystrophin-positive, called sarcoglycanopathies or limb girdle muscular dystrophies. Our recent studies have demonstrated that costameres are a proteic machinery made up of DGC and vinculin-talin-integrin system, also revealing the colocalization of sarcoglycans and integrins in adult human skeletal muscle. These results may support the hypothesis of the existence of a bidirectional signalling between sarcoglycans and integrins in cultured L6 myocytes. The hypothesis of bidirectional signalling between sarcoglycans and integrins could be supported by the identification of a skeletal and cardiac muscle filamin2 as a γ-sarcoglycan, δ-sarcoglycan and, hypothetically, β1 integrin interacting protein. Our results, acquired with an immunofluorescence study on adult human skeletal muscle affected by LGMD type 2D and 2C, showed that in LGMD2D: a) α-sarcoglycan staining is severely reduced; b) the β-γ-δ-sarcoglycan subunit and all tested integrins staining are clearly detectable; c) filamin2 is normal and shows a costameric distribution. In LGMD2C: a) α-sarcoglycan staining is preserved; b) the β-γ-δ-sarcoglycan subunit staining is severely reduced; c) the α7B-integrin is slightly reduced and β1D-integrin is severely reduced; d) filamin2 is severely reduced. Other tested proteins of the two systems show a normal staining pattern in both sarcoglycanopathies. Our study seems to confirm, for the first time on adult human skeletal muscle of subjects affected by LGMDs, the hypo-theses of: a) the existence, in mouse myotubes in culture, of two distinct subunits in sarcoglycans subcomplex; b) the presence of a bidirectional signalling between sarcoglycans and integrins, previously demonstrated on rat cultured L6 myocytes; c) the interaction of FLN2 with both sarcoglycans and integrins. These results may stimulate the search of yet unidentified common interactors of both fiber-extracellular matrix interaction systems.

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December 2004
Volume 14 Issue 6

Print ISSN: 1107-3756
Online ISSN:1791-244X

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Spandidos Publications style
Anastasi G, Cutroneo G, Trimarchi F, Santoro G, Bruschetta D, Bramanti P, Pisani A and Favaloro A: Evaluation of sarcoglycans, vinculin-talin-integrin system and filamin2 in α- and γ-sarcoglycanopathy: An immunohistochemical study. Int J Mol Med 14: 989-999, 2004
APA
Anastasi, G., Cutroneo, G., Trimarchi, F., Santoro, G., Bruschetta, D., Bramanti, P. ... Favaloro, A. (2004). Evaluation of sarcoglycans, vinculin-talin-integrin system and filamin2 in α- and γ-sarcoglycanopathy: An immunohistochemical study. International Journal of Molecular Medicine, 14, 989-999. https://doi.org/10.3892/ijmm.14.6.989
MLA
Anastasi, G., Cutroneo, G., Trimarchi, F., Santoro, G., Bruschetta, D., Bramanti, P., Pisani, A., Favaloro, A."Evaluation of sarcoglycans, vinculin-talin-integrin system and filamin2 in α- and γ-sarcoglycanopathy: An immunohistochemical study". International Journal of Molecular Medicine 14.6 (2004): 989-999.
Chicago
Anastasi, G., Cutroneo, G., Trimarchi, F., Santoro, G., Bruschetta, D., Bramanti, P., Pisani, A., Favaloro, A."Evaluation of sarcoglycans, vinculin-talin-integrin system and filamin2 in α- and γ-sarcoglycanopathy: An immunohistochemical study". International Journal of Molecular Medicine 14, no. 6 (2004): 989-999. https://doi.org/10.3892/ijmm.14.6.989