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פותח על ידי קלירמאש פתרונות בע"מ -
Flicker cone function in normal and day blind sheep: a large animal model for human achromatopsia caused by CNGA3 mutation
Year:
2014
Source of publication :
Documenta Ophthalmologica
Authors :
גוטויין, אלישע
;
.
Volume :
129
Co-Authors:
Ezra-Elia, R., Koret School of Veterinary Medicine, Hebrew University of Jerusalem, PO Box 12, Rehovot, Israel
Banin, E., Department of Ophthalmology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel
Honig, H., Agricultural Research Organization, The Volcani Center, Bet Dagan, Israel
Rosov, A., Agricultural Research Organization, The Volcani Center, Bet Dagan, Israel
Obolensky, A., Department of Ophthalmology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel
Averbukh, E., Department of Ophthalmology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel
Hauswirth, W.W., Department of Ophthalmology, University of Florida, Gainesville, FL, United States
Gootwine, E., Agricultural Research Organization, The Volcani Center, Bet Dagan, Israel
Ofri, R., Koret School of Veterinary Medicine, Hebrew University of Jerusalem, PO Box 12, Rehovot, Israel
Facilitators :
From page:
141
To page:
150
(
Total pages:
10
)
Abstract:
Purpose: Recently we reported on day blindness in sheep caused by a mutation in the CNGA3 gene, thus making affected sheep a naturally occurring large animal model for therapeutic intervention in CNGA3 achromatopsia patients. The purpose of this study was to characterize flicker cone function in normal and day blind sheep, with the aim of generating a normative data base for ongoing gene therapy studies.Methods: Electoretinographic (ERG) cone responses were evoked with full-field conditions in 10 normal, 6 heterozygous carriers and 36 day blind sheep. Following light adaptation (10 min, 30 cd/m2), responses were recorded at four increasing light intensities (1, 2.5, 5 and 10 cd s/m2). At each of these intensities, a single photopic flash response followed by 8 cone flicker responses (10–80 Hz) was recorded. Results were used to generate a normative data base for the three groups. Differences between day blind and normal control animals were tested in two age-matched groups (n = 10 per group).Results: The normal sheep cone ERG wave is bipartite in nature, with critical flicker fusion frequency (CFF) >80 Hz. In all four flash intensities, the single photopic flash a-wave and b-wave amplitudes were significantly lower (p < 0.005), and implicit times significantly delayed (p < 0.0001), in day blind animals. In all four flash intensities, CFF values were significantly lower (p < 0.0001) in day blind sheep.Conclusions: Cone function is severely depressed in day blind sheep. Our results will provide a normative data base for ongoing gene therapy studies. © 2014, Springer-Verlag Berlin Heidelberg.
Note:
Related Files :
Animal
animal experiment
Animals
Female
gene therapy
Genetics
Male
mutation
sheep
עוד תגיות
תוכן קשור
More details
DOI :
10.1007/s10633-014-9458-6
Article number:
Affiliations:
Database:
סקופוס
Publication Type:
מאמר
;
.
Language:
אנגלית
Editors' remarks:
ID:
23468
Last updated date:
02/03/2022 17:27
Creation date:
16/04/2018 23:59
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Scientific Publication
Flicker cone function in normal and day blind sheep: a large animal model for human achromatopsia caused by CNGA3 mutation
129
Ezra-Elia, R., Koret School of Veterinary Medicine, Hebrew University of Jerusalem, PO Box 12, Rehovot, Israel
Banin, E., Department of Ophthalmology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel
Honig, H., Agricultural Research Organization, The Volcani Center, Bet Dagan, Israel
Rosov, A., Agricultural Research Organization, The Volcani Center, Bet Dagan, Israel
Obolensky, A., Department of Ophthalmology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel
Averbukh, E., Department of Ophthalmology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel
Hauswirth, W.W., Department of Ophthalmology, University of Florida, Gainesville, FL, United States
Gootwine, E., Agricultural Research Organization, The Volcani Center, Bet Dagan, Israel
Ofri, R., Koret School of Veterinary Medicine, Hebrew University of Jerusalem, PO Box 12, Rehovot, Israel
Flicker cone function in normal and day blind sheep: a large animal model for human achromatopsia caused by CNGA3 mutation
Purpose: Recently we reported on day blindness in sheep caused by a mutation in the CNGA3 gene, thus making affected sheep a naturally occurring large animal model for therapeutic intervention in CNGA3 achromatopsia patients. The purpose of this study was to characterize flicker cone function in normal and day blind sheep, with the aim of generating a normative data base for ongoing gene therapy studies.Methods: Electoretinographic (ERG) cone responses were evoked with full-field conditions in 10 normal, 6 heterozygous carriers and 36 day blind sheep. Following light adaptation (10 min, 30 cd/m2), responses were recorded at four increasing light intensities (1, 2.5, 5 and 10 cd s/m2). At each of these intensities, a single photopic flash response followed by 8 cone flicker responses (10–80 Hz) was recorded. Results were used to generate a normative data base for the three groups. Differences between day blind and normal control animals were tested in two age-matched groups (n = 10 per group).Results: The normal sheep cone ERG wave is bipartite in nature, with critical flicker fusion frequency (CFF) >80 Hz. In all four flash intensities, the single photopic flash a-wave and b-wave amplitudes were significantly lower (p < 0.005), and implicit times significantly delayed (p < 0.0001), in day blind animals. In all four flash intensities, CFF values were significantly lower (p < 0.0001) in day blind sheep.Conclusions: Cone function is severely depressed in day blind sheep. Our results will provide a normative data base for ongoing gene therapy studies. © 2014, Springer-Verlag Berlin Heidelberg.
Scientific Publication
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