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Molecular cloning and protein expression of Duchenne muscular dystrophy gene products in porcine retina.

Abstract : Due to the difference between rodent and human retinal circuitry, we characterize a new animal model of retinal perturbation in neurotransmission in Duchenne Muscular Dystrophy (DMD) patients. We investigated the expression and localization of dystrophin proteins and dystrophin associated proteins in porcine retina by reverse transcription polymerase chain reaction, Western blot analysis and immunohistochemistry. Homologues of human DMD gene products and alternative spliced isoforms of Dp71 were identified. We observed that dystrophins were expressed in the outer plexiform layer, around blood vessels and at the inner limiting membrane as previously described in human and mouse retinae. Moreover, by double immunostaining we showed that beta-dystroglycan co-localizes with dystrophin in the outer plexiform layer whereas alpha1-syntrophin labeling differs from that for dystrophins. Using confocal laser microscopy we observed that dystrophins labeling co-localizes with pre- and post-synaptic cell markers in the outer plexiform layer. We suggest that porcine retina constitutes a good model to study the role of dystrophins in retinal neurotransmission and should be used to investigate the physiological roles of dystrophins in signal transduction.
Keywords : Dystrophins DAPs Retina
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Submitted on : Sunday, May 31, 2020 - 9:10:48 PM
Last modification on : Monday, July 19, 2021 - 5:52:03 PM

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Agnès Bordais, Francisco Bolaños-Jiménez, Patrice Fort, Carolina Varela, José Alain Sahel, et al.. Molecular cloning and protein expression of Duchenne muscular dystrophy gene products in porcine retina.. Neuromuscular Disorders, Elsevier, 2005, 15 (7), pp.476-487. ⟨10.1016/j.nmd.2005.03.011⟩. ⟨hal-02679136⟩

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