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Thomas Claudepierre, Dominique Mornet, Thomas Pannicke, Valérie Forster, Cécile Dalloz, Francisco Bolaños, José Sahel, Andreas Reichenbach, Alvaro Rendon; Expression of Dp71 in Müller Glial Cells: A Comparison with Utrophin- and Dystrophin-Associated Proteins. Invest. Ophthalmol. Vis. Sci. 2000;41(1):294-304.
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© ARVO (1962-2015); The Authors (2016-present)
purpose. The abnormal retinal electrophysiology observed in patients with
Duchenne muscular dystrophy (DMD) has been attributed to an altered
expression of C-terminal products of the dystrophin gene. It has been
shown that Dp260 is expressed by photoreceptor cells, whereas Dp71 is
present in glial cells. The present study was intended to identify all
known members of the dystrophin superfamily and their associated
proteins expressed in Müller glial cells (MGC).
methods. The expression of the proteins and of their messengers was studied in
MGC cultures from 2-week-old rats, by polymerase chain reaction
amplification, Western blot analysis, and immunocytochemistry. An
immunocytochemical localization of the proteins was also performed on
enzymatically dissociated Müller cells from adult rat retinas.
results. MGCs expressed a spliced isoform of Dp71 called Dp71f, as well as
utrophin, β-dystroglycan, δ- and γ-sarcoglycans, andα
1-syntrophin. In morphologically preserved differentiated
Müller cells, Dp71f was localized in clusters, utrophin was
diffusely distributed in the cytoplasm, and dystrophin-associated
proteins (DAPs) were membrane-bound. Most of these proteins were
preferentially expressed in the vitread portion of the cells. Dp71f and
utrophin expression was restricted to MGCs, whereas all DAPs were also
present in other retinal cell types.
conclusions. The exclusive localization of Dp71f and utrophin in MGCs suggests that
these proteins, together with DAPs, play a specific role in these
cells. Further knowledge of possible interactions of these proteins
within a functional complex may provide new insights into the molecular
basis of the electroretinogram phenotype in DMD.
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