title: Retinal pigment epithelium protein of 65 kDA gene-linked retinal degeneration is not modulated by chicken acidic leucine-rich epidermal growth factor-like domain containing brain protein/Neuroglycan C/ chondroitin sulfate proteoglycan 5.
abstract: OBJECTIVE
  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 α subunit (Gnat1), and cone transducin α 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.
authors.affiliation: Institute for Research in Ophthalmology, Sion, Switzerland ; Department of Ophthalmology, University of Lausanne, Lausanne, Switzerland.
authors.forename: Sandra
authors.initials: S
authors.lastname: Cottet
authors.forename: René
authors.initials: R
authors.lastname: Jüttner
authors.forename: Nathalie
authors.initials: N
authors.lastname: Voirol
authors.forename: Pierre
authors.initials: P
authors.lastname: Chambon
authors.forename: Fritz G
authors.initials: FG
authors.lastname: Rathjen
authors.forename: Daniel F
authors.initials: DF
authors.lastname: Schorderet
authors.forename: Pascal
authors.initials: P
authors.lastname: Escher
mesh_terms: D000818:Animals; D005786:Gene Expression Regulation; D008565:Membrane Proteins / Q000172:deficiency / Q000378:metabolism*; D051379:Mice; D009928:Organ Specificity / Q000235:genetics; D011509:Proteoglycans / Q000172:deficiency / Q000378:metabolism*; D017949:Retinal Cone Photoreceptor Cells / Q000378:metabolism / Q000473:pathology; D012162:Retinal Degeneration / Q000235:genetics* / Q000473:pathology; D017948:Retinal Rod Photoreceptor Cells / Q000378:metabolism / Q000473:pathology; D013997:Time Factors; D019745:cis-trans-Isomerases / Q000172:deficiency / Q000235:genetics* / Q000378:metabolism; D000099178:Retinoid Isomerohydrolase
chemical_list: D008565:Membrane Proteins; D011509:Proteoglycans; D019745:cis-trans-Isomerases; D000099178:Retinoid Isomerohydrolase; C403173:Cspg5 protein, mouse; C108001:Stra6 protein, mouse