| Structural and Functional Phenotyping in the Cone-Specific Photoreceptor Function Loss 1 (cpfl1) Mouse Mutant - A Model of Cone Dystrophies. | |
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MedLine Citation:
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PMID: 20238063 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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Purpose: We performed a comprehensive in vivo assessment of retinal morphology and function in cpfl1 (cone photoreceptor function loss 1) mice to better define the disease process in this model of cone dystrophies. Methods: Mice were examined using electroretinography (ERG), confocal scanning laser ophthalmoscopy (cSLO), and spectral domain optical coherence tomography (SD-OCT). Cross-breeding cpfl1 mutants with mice expressing green fluorescent protein (GFP) under control of red-green cone opsin promoter allowed for an in vivo timeline analysis of number and distribution of cone photoreceptors using the autofluorescence (AF) mode of the cSLO. Results: Light-evoked responses of cone origin were practically absent in cpfl1 mice, whereas rod system function appeared normal. In vivo imaging revealed a progressive loss of cone photoreceptors with a major decline between PW4 and PW8, while retinal architecture and layering remained essentially intact. Discussion: While the absence of substantial light-evoked cone responses in the cpfl1 mice is evident from early on, the course of physical cone degeneration is protracted and has a major drop between PW4 and PW8. However, these changes do not lead to significant alterations in retinal architecture, probably due to the relatively low number and wide dissemination of cone photoreceptor cells within the afoveate mouse retina. |
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Authors:
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M Dominik Fischer; Naoyuki Tanimoto; Susanne C Beck; Gesine Huber; Karin Schaeferhoff; Stylianos Michalakis; Olaf Riess; Bernd Wissinger; Martin Biel; Michael Bonin; Mathias W Seeliger |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Advances in experimental medicine and biology Volume: 664 ISSN: 0065-2598 ISO Abbreviation: Adv. Exp. Med. Biol. Publication Date: 2010 |
Date Detail:
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Created Date: 2010-03-18 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0121103 Medline TA: Adv Exp Med Biol Country: United States |
Other Details:
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Languages: eng Pagination: 593-9 Citation Subset: IM |
Affiliation:
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Division of Ocular Neurodegeneration, Institute for Ophthalmic Research, Centre for Ophthalmology, University of Tuebingen, Schleichstr. 12-16, 72070, Tuebingen, Germany, dominik.fischer@med.uni-tuebingen.de. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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