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The sarcoglycan-sarcospan complex localization in mouse retina is independent from dystrophins.

Abstract : The sarcoglycan-sarcospan (SG-SSPN) complex is part of the dystrophin-glycoprotein complex that has been extensively characterized in muscle. To establish the framework for functional studies of sarcoglycans in retina here, we quantified sarcoglycans mRNA levels with real-time reverse transcriptase-polymerase chain reaction (RT-PCR) and performed immunohistochemistry to determine their cellular and subcellular distribution. We showed that the beta-, delta-, gamma-, epsilon-sarcoglycans and sarcospan are expressed in mouse retina. They are localized predominantly in the outer and the inner limiting membranes, probably in the M?r cells and also in the ganglion cells axons where the expression of dystrophins have never been reported. We also investigated the status of the sarcoglycans in the retina of mdx(3cv) mutant mice for all Duchene Muscular Dystrophy (DMD) gene products. The absence of dystrophin did not produce any change in the sarcoglycan-sarcospan components expression and distribution.
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Contributor : Dominique Mornet <>
Submitted on : Wednesday, May 23, 2007 - 3:38:23 PM
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Patrice Fort, Francisco-Javier Estrada, Agnès Bordais, Dominique Mornet, José-Alain Sahel, et al.. The sarcoglycan-sarcospan complex localization in mouse retina is independent from dystrophins.. Neuroscience Research, Elsevier, 2005, 53 (1), pp.25-33. ⟨10.1016/j.neures.2005.05.007⟩. ⟨inserm-00148784⟩



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