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

The sarcoglycan-sarcospan complex localization in mouse retina is independent from dystrophins. - Descarga este documento en PDF. Documentación en PDF para descargar gratis. Disponible también para leer online.

* Corresponding author 1 Laboratoire de Physiopathologie Cellulaire et Moleculaire de la Retine 2 Unidad de Investigacion Medica en Genetica Humana 3 Molecular Biology Laboratory 4 Muscles et pathologies chroniques

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 mdx3cv 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.

Autor: Patrice Fort - Francisco-Javier Estrada - Agnès Bordais - Dominique Mornet - José-Alain Sahel - Serge Picaud - Haydeé Rosas Va



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