Structural and functional phenotyping in the cone-specific photoreceptor function loss 1 (cpfl1) mouse mutant - a model of cone dystrophies.
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...
| Main Authors: | , , , , , , , , , , |
|---|---|
| Format: | Journal article |
| Language: | English |
| Published: |
2010
|
| _version_ | 1797105006477836288 |
|---|---|
| author | Fischer, M Tanimoto, N Beck, S Huber, G Schaeferhoff, K Michalakis, S Riess, O Wissinger, B Biel, M Bonin, M Seeliger, M |
| author_facet | Fischer, M Tanimoto, N Beck, S Huber, G Schaeferhoff, K Michalakis, S Riess, O Wissinger, B Biel, M Bonin, M Seeliger, M |
| author_sort | Fischer, M |
| collection | OXFORD |
| description | 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. |
| first_indexed | 2024-03-07T06:41:22Z |
| format | Journal article |
| id | oxford-uuid:f9659b85-13ad-4fc9-8357-574ab9cef3bf |
| institution | University of Oxford |
| language | English |
| last_indexed | 2024-03-07T06:41:22Z |
| publishDate | 2010 |
| record_format | dspace |
| spelling | oxford-uuid:f9659b85-13ad-4fc9-8357-574ab9cef3bf2022-03-27T12:57:39ZStructural and functional phenotyping in the cone-specific photoreceptor function loss 1 (cpfl1) mouse mutant - a model of cone dystrophies.Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:f9659b85-13ad-4fc9-8357-574ab9cef3bfEnglishSymplectic Elements at Oxford2010Fischer, MTanimoto, NBeck, SHuber, GSchaeferhoff, KMichalakis, SRiess, OWissinger, BBiel, MBonin, MSeeliger, M 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. |
| spellingShingle | Fischer, M Tanimoto, N Beck, S Huber, G Schaeferhoff, K Michalakis, S Riess, O Wissinger, B Biel, M Bonin, M Seeliger, M Structural and functional phenotyping in the cone-specific photoreceptor function loss 1 (cpfl1) mouse mutant - a model of cone dystrophies. |
| title | Structural and functional phenotyping in the cone-specific photoreceptor function loss 1 (cpfl1) mouse mutant - a model of cone dystrophies. |
| title_full | Structural and functional phenotyping in the cone-specific photoreceptor function loss 1 (cpfl1) mouse mutant - a model of cone dystrophies. |
| title_fullStr | Structural and functional phenotyping in the cone-specific photoreceptor function loss 1 (cpfl1) mouse mutant - a model of cone dystrophies. |
| title_full_unstemmed | Structural and functional phenotyping in the cone-specific photoreceptor function loss 1 (cpfl1) mouse mutant - a model of cone dystrophies. |
| title_short | Structural and functional phenotyping in the cone-specific photoreceptor function loss 1 (cpfl1) mouse mutant - a model of cone dystrophies. |
| title_sort | structural and functional phenotyping in the cone specific photoreceptor function loss 1 cpfl1 mouse mutant a model of cone dystrophies |
| work_keys_str_mv | AT fischerm structuralandfunctionalphenotypingintheconespecificphotoreceptorfunctionloss1cpfl1mousemutantamodelofconedystrophies AT tanimoton structuralandfunctionalphenotypingintheconespecificphotoreceptorfunctionloss1cpfl1mousemutantamodelofconedystrophies AT becks structuralandfunctionalphenotypingintheconespecificphotoreceptorfunctionloss1cpfl1mousemutantamodelofconedystrophies AT huberg structuralandfunctionalphenotypingintheconespecificphotoreceptorfunctionloss1cpfl1mousemutantamodelofconedystrophies AT schaeferhoffk structuralandfunctionalphenotypingintheconespecificphotoreceptorfunctionloss1cpfl1mousemutantamodelofconedystrophies AT michalakiss structuralandfunctionalphenotypingintheconespecificphotoreceptorfunctionloss1cpfl1mousemutantamodelofconedystrophies AT riesso structuralandfunctionalphenotypingintheconespecificphotoreceptorfunctionloss1cpfl1mousemutantamodelofconedystrophies AT wissingerb structuralandfunctionalphenotypingintheconespecificphotoreceptorfunctionloss1cpfl1mousemutantamodelofconedystrophies AT bielm structuralandfunctionalphenotypingintheconespecificphotoreceptorfunctionloss1cpfl1mousemutantamodelofconedystrophies AT boninm structuralandfunctionalphenotypingintheconespecificphotoreceptorfunctionloss1cpfl1mousemutantamodelofconedystrophies AT seeligerm structuralandfunctionalphenotypingintheconespecificphotoreceptorfunctionloss1cpfl1mousemutantamodelofconedystrophies |