Glial dystrophin-associated proteins, laminin and agrin, are downregulated in the brain of mdx mouse

Beatrice Nico, Roberto Tamma, Tiziana Annese, Domenica Mangieri, Annamaria De Luca, Patrizia Corsi, Vincenzo Benagiano, Vito Longo, Enrico Crivellato, Andrea Salmaggi, Domenico Ribatti

Research output: Contribution to journalArticlepeer-review


In this study, we investigated the involvement of dystrophin-associated proteins (DAPs) and their relationship with the perivascular basement membrane in the brains of mdx mice and controls at the age of 2 months. We analyzed (1) the expression of glial DAPs α-Β-dystroglycan (DG), α-syntrophin, aquaporin-4 (AQP4) water channel, Kir 4.1 and dystrophin isoform (Dp71) by immunocytochemistry, laser confocal microscopy, immunogold electron microscopy, immunoblotting and RT-PCR; (2) the ultrastructure of the basement membrane and expression of laminin and agrin; and (3) the dual immunofluorescence colocalization of AQP4/α-Β-DG, and of Kir 4.1/agrin. The following results were observed in mdx brain as compared with controls: (1) a significant reduction in protein content and mRNA expression of DAPs; (2) ultrastructurally, a thickened and discontinuous appearance of the basement membrane and a significant reduction in laminin and agrin; and (3) a molecular rearrangment of α-Β-DG, coupled with a parallel loss of agrin and Kir 4.1 on basement membrane and glial endfeet. These data indicate that in mdx brain the deficiency in dystrophin and dystrophin isoform (Dp71) is coupled with a reduction of DAP components, coupled with an altered anchoring to the basement membrane.

Original languageEnglish
Pages (from-to)1645-1660
Number of pages16
JournalLaboratory Investigation
Issue number11
Publication statusPublished - Nov 2010


  • astrocyte
  • basement membrane
  • blood-brain barrier
  • DAPs
  • dystrophy

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Cell Biology
  • Molecular Biology


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