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| Item Type: | Article |
|---|---|
| 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 |
| Creators Name: | Cottet, S., Jüttner, R., Voirol, N., Chambon, P., Rathjen, F.G., Schorderet, D.F. and Escher, P. |
| Abstract: | 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. |
| Keywords: | Gene Expression Regulation, Membrane Proteins, Organ Specificity, Proteoglycans, Retinal Cone Photoreceptor Cells, Retinal Degeneration, Retinal Rod Photoreceptor Cells, Time Factors, Cis-Trans-Isomerases, Animals, Mice |
| Source: | Molecular Vision |
| ISSN: | 1090-0535 |
| Publisher: | Molecular Vision |
| Volume: | 19 |
| Page Range: | 2312-2320 |
| Date: | 16 November 2013 |
| Official Publication: | http://www.molvis.org/molvis/v19/2312 |
| PubMed: | View item in PubMed |
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