Dp71 expression in human glioblastoma

Simona Ruggieri, Michelina De Giorgis, Tiziana Annese, Roberto Tamma, Angelo Notarangelo, Andrea Marzullo, Rebecca Senetta, Paola Cassoni, Michela Notarangelo, Domenico Ribatti, Beatrice Nico

Research output: Contribution to journalArticle

Abstract

Background: Dp71 is the most abundant dystrophin (DMD) gene product in the nervous system. Mutation in the Dp71 coding region is associated with cognitive disturbances in Duchenne muscular dystrophy (DMD) patients, but the function of dystrophin Dp71 in tumor progression remains to be established. This study investigated Dp71 expression in glioblastoma, the most common and aggressive primary tumor of the central nervous system (CNS). Methods: Dp71 expression was analyzed by immunofluorescence, immunohistochemistry, RT-PCR, and immunoblotting in glioblastoma cell lines and cells isolated from human glioblastoma multiforme (GBM) bioptic specimens. Results: Dp71 isoform was expressed in normal human astrocytes (NHA) cell lines and decreased in glioblastoma cell lines and cells isolated from human glioblastoma multiforme bioptic specimens. Moreover, Dp71 was localized in the nucleus in normal cells, while it was localized into the cytoplasm of glioblastoma cells organized in clusters. We have shown, by double labeling, that Dp71 colocalizes with lamin B in normal astrocytes cells, confirming the roles of Dp71 and lamin B in maintaining nuclear architecture. Finally, we demonstrated that decreased Dp71 protein in cells isolated from human bioptic specimens was inversely correlated with the Ki-67 tumor proliferative index. Conclusion: A decreased Dp71 expression is associated with cancer proliferation and poor prognosis in glioblastoma.

Original languageEnglish
Article number5429
JournalInternational Journal of Molecular Sciences
Volume20
Issue number21
DOIs
Publication statusPublished - Nov 1 2019

Keywords

  • Dp71
  • Glioblastoma
  • Lamin B
  • Tumor progression

ASJC Scopus subject areas

  • Catalysis
  • Molecular Biology
  • Spectroscopy
  • Computer Science Applications
  • Physical and Theoretical Chemistry
  • Organic Chemistry
  • Inorganic Chemistry

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  • Cite this

    Ruggieri, S., De Giorgis, M., Annese, T., Tamma, R., Notarangelo, A., Marzullo, A., Senetta, R., Cassoni, P., Notarangelo, M., Ribatti, D., & Nico, B. (2019). Dp71 expression in human glioblastoma. International Journal of Molecular Sciences, 20(21), [5429]. https://doi.org/10.3390/ijms20215429