title: 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.
authors.affiliation: Laboratoire de Physiopathologie Cellulaire et Moléculaire de la Rétine, INSERM U592, Hôpital Saint-Antoine, Bâtiment Kourilsky, 184 rue du Faubourg Saint-Antoine, 75571 Paris, France.
authors.forename: Agnès
authors.initials: A
authors.lastname: Bordais
authors.forename: Francisco
authors.initials: F
authors.lastname: Bolaños-Jimenez
authors.forename: Patrice
authors.initials: P
authors.lastname: Fort
authors.forename: Carolina
authors.initials: C
authors.lastname: Varela
authors.forename: José-Alain
authors.initials: JA
authors.lastname: Sahel
authors.forename: Serge
authors.initials: S
authors.lastname: Picaud
authors.forename: Alvaro
authors.initials: A
authors.lastname: Rendon
mesh_terms: D017398:Alternative Splicing; D000818:Animals; D015153:Blotting, Western / Q000379:methods; D002135:Calcium-Binding Proteins / Q000378:metabolism; D003001:Cloning, Molecular; D049030:Dystroglycans / Q000378:metabolism; D016189:Dystrophin / Q000235:genetics* / Q000378:metabolism; D049029:Dystrophin-Associated Proteins / Q000235:genetics / Q000378:metabolism; D005786:Gene Expression Regulation / Q000502:physiology*; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D008565:Membrane Proteins / Q000378:metabolism; D051379:Mice; D009124:Muscle Proteins / Q000378:metabolism; D020388:Muscular Dystrophy, Duchenne / Q000235:genetics* / Q000378:metabolism; D012333:RNA, Messenger / Q000378:metabolism; D012160:Retina / Q000378:metabolism*; D020133:Reverse Transcriptase Polymerase Chain Reaction / Q000379:methods; D017421:Sequence Analysis / Q000379:methods; D013552:Swine / Q000235:genetics*
chemical_list: D002135:Calcium-Binding Proteins; D016189:Dystrophin; D049029:Dystrophin-Associated Proteins; D008565:Membrane Proteins; D009124:Muscle Proteins; D012333:RNA, Messenger; C091825:syntrophin alpha1; D049030:Dystroglycans