Annese, Tiziana

Benagiano, Vincenzo

Crivellato, Enrico

Longo, Vito

Mangieri, Domenica

Nico, Beatrice

Ribatti, Domenico

Tamma, Roberto

Firenze University Press: E-Journals

IJAE Vol. 115, n. 1/2 (Supplement), 2010
163
Role of glial dystrophin-associated proteins and 
perivascular basement membrane in blood brain 
barrier alteration of mdx mouse
Roberto Tamma1, Tiziana Annese1, Domenica Mangieri2, Vincenzo Benagiano2, Vito Longo3, Enrico 
Crivellato4, Domenico Ribatti1, Beatrice Nico1
1 Department of Human Anatomy and Histology University of Bari Medical School, Italy
2 Department of Clinical Medicine, Nephrology and Health Sciences, University of Parma Medical School, Italy
3 Department ofInternal Medicine and Clinical Oncology, University of Bari Medical School, Italy
4 Department of Medical and Morphological Research, Anatomy Section, University of Udine Medical School, Italy
In this study we have investigated the involvement of the dystrophin–associated 
proteins (DAPs) and their relationship with the perivascular basement membrane in 
the brain of two months mdx mice and in the control ones. We have analyzed: 1) The 
expression of the glial DAPs α-β dystroglycan, -syntrophyn, aquaporin-4 water chan-
nel (AQP4), Kir 4.1 and Dp71, by immunocytochemistry, laser confocal microscopy, im-
munogold electron microscopy, immunoblotting and RT-PCR; 2) The ultrastructure of 
the basement membrane and the expression of laminin and agrin; 3) The dual immun-
ofluorescence colocalization of AQP4/α-β dystroglycan, and of Kir-4.1/agrin. Results 
showed in mdx brain as compared to control ones: 1) A significant reduction in protein 
contents and mRNA expression of DAPs; 2) Ultrastructurally, the basement membrane 
appeared thickened and discontinuous and showed a significant reduction in laminin 
and agrin; 3) A molecular rearrangment of the α-β dystroglycan, 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 Dp71 is coupled with a reduction of 
the DAPs components coupled with an altered anchoring to the basement membrane.


Role of glial dystrophin-associated proteins and perivascular basement membrane in blood brain barrier alteration of mdx mouse

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Role of glial dystrophin-associated proteins and perivascular basement membrane in blood brain barrier alteration of mdx mouse

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