Volz, Cornelia, Mirza, Myriam, Langmann, Thomas and Jaegle, Herbert (2014). Retinal Function in Aging Homozygous Knock-In Mice. S. 495 - 502. BERLIN: SPRINGER-VERLAG BERLIN. ISSN 2214-8019
Full text not available from this repository.Abstract
Neuronal ceroid lipofuscinoses (NCL) are characterized by lysosomal accumulation of autofluorescent material and lead to degeneration of the central nervous system. Patients affected by the juvenile form of NCL (JNCL), the most common form of the disease, develop visual failure prior to mental and motor deficits. It is currently unclear if the corresponding mouse model, Cln3(Delta ex7/8) knock-in, develops the same retinal phenotype and electroretinogram (ERG) measurements as affected patients. The aim of our study was to investigate the visual disease progression in the Cln3(Delta ex7/8) mice using scotopic and photopic ERGs as well as optokinetic tracking (OKT) at different ages. The results were then compared with age-matched controls. The amplitudes of the a-wave and b-wave (scotopic ERG) decrease significantly in Cln3(Delta ex7/8) mice starting at the age of 12 months. A reduction in the b/a-amplitude ratio indicates a degeneration preferentially of the inner retina. An amplitude reduction observed in the Cln3(+/+) control mice may be attributed to an additional Crb1(rd8) mutation. Using optokinetic tracking (OKT) the Cln3(Delta ex7/8) mice show a progressive decline in visual acuity after 12 months of age.
Item Type: | Journal Article | ||||||||||||||||||||
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URN: | urn:nbn:de:hbz:38-448999 | ||||||||||||||||||||
DOI: | 10.1007/978-1-4614-3209-8_63 | ||||||||||||||||||||
Title of Book: | Advances in Experimental Medicine and Biology | ||||||||||||||||||||
Series Name: | Adv.Exp.Med.Biol. | ||||||||||||||||||||
Volume: | 801 | ||||||||||||||||||||
Page Range: | S. 495 - 502 | ||||||||||||||||||||
Date: | 2014 | ||||||||||||||||||||
Publisher: | SPRINGER-VERLAG BERLIN | ||||||||||||||||||||
Place of Publication: | BERLIN | ||||||||||||||||||||
ISSN: | 2214-8019 | ||||||||||||||||||||
ISBN: | 978-1-4614-3209-8; 978-1-4614-3208-1 | ||||||||||||||||||||
Language: | English | ||||||||||||||||||||
Faculty: | Unspecified | ||||||||||||||||||||
Divisions: | Unspecified | ||||||||||||||||||||
Subjects: | no entry | ||||||||||||||||||||
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URI: | http://kups.ub.uni-koeln.de/id/eprint/44899 |
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