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A. Rendon, J. Estrada–Mena, A. Bordais, J.A. Sahel, R.M. Coral–Vazquez, H. Rosas–Vargas, P.E. Fort; Identification and Localization of the Constituents of the Sarcoglycan Complex in Retinae From Wild–Type and Dystrophins Deficient Mice (mdx3cv) . Invest. Ophthalmol. Vis. Sci. 2005;46(13):2991.
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© ARVO (1962-2015); The Authors (2016-present)
Purpose: Sarcoglycans (SGs) are essential constituents of the dystrophin–associated protein (DAPs) complex, a large membrane–associated protein scaffold that is critical for the anchoring and localization of the Acetylcholine receptor at the neuromuscular junction. The SGs are associated with dystrophins by the intermediary of the dystroglycan complex. Since dystrophins and dystroglycans are expressed in mouse retinae, here we analyzed the SGs expression and localization in wild–type (wt) and dystrophins deficient mice retinae (mdx3cv). Methods: The expression and localization of dystrophins, utrophins and sarcoglycans were determined by Real–time RT–PCR and immunocytochemistry methods in retinae of wild–type and mdx3cv mice strains with specific antibodies. All fluorescence specimens were viewed by confocal microscopy. Results: We found that ß–, Δ–, γ–, ε– SGs and sarcospan are expressed at various levels in wt mice retinae. α–sarcoglycan a typical constituent of SGs complex in skeletal muscle is not expressed. Immunolocalization revealed that sarcoglycans were mainly concentrated in the ganglion cell layer and at the level of the outer limiting membrane. Using mdx3cv mice strain we showed that absence of dystrophins has no effect on the expression and localization of the SGs. Conclusions: Our results suggest that SGs complex(es) exist in retina but they are not dystrophin–dependant.
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