Fiche publication
Date publication
janvier 2013
Auteurs
Membres identifiés du Cancéropôle Est :
Pr CHAMBON Pierre
Tous les auteurs :
Cottet S, Juttner R, Voirol N, Chambon P, Rathjen FG, Schorderet DF, Escher P
Lien Pubmed
Résumé
PURPOSE: To analyze in vivo the function of chicken acidic leucine-rich epidermal growth factor-like domain containing brain protein/Neuroglycan C (gene symbol: Cspg5) during retinal degeneration in the Rpe65(-)/(-) mouse model of Leber congenital amaurosis. METHODS: We resorted to mice with targeted deletions in the Cspg5 and retinal pigment epithelium protein of 65 kDa (Rpe65) genes (Cspg5(-)/(-)/Rpe65(-)/(-)). Cone degeneration was assessed with cone-specific peanut agglutinin staining. Transcriptional expression of rhodopsin (Rho), S-opsin (Opn1sw), M-opsin (Opn1mw), rod transducin alpha subunit (Gnat1), and cone transducin alpha subunit (Gnat2) genes was assessed with quantitative PCR from 2 weeks to 12 months. The retinal pigment epithelium (RPE) was analyzed at P14 with immunodetection of the retinol-binding protein membrane receptor Stra6. RESULTS: No differences in the progression of retinal degeneration were observed between the Rpe65(-)/(-) and Cspg5(-)/(-)/Rpe65(-)/(-) mice. No retinal phenotype was detected in the late postnatal and adult Cspg5(-)/(-) mice, when compared to the wild-type mice. CONCLUSIONS: Despite the previously reported upregulation of Cspg5 during retinal degeneration in Rpe65(-)/(-) mice, no protective effect or any involvement of Cspg5 in disease progression was identified.
Référence
Mol Vis. 2013 Nov 16;19:2312-20. eCollection 2013.
