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

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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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