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lüll Retinal function in aging homozygous Cln3 (Deltaex7/8) knock-in mice Volz C; Mirza M; Langmann T; Jagle HAdv Exp Med Biol 2014[]; 801 (ä): 495-501Neuronal 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 (Deltaex7/8) 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 (Deltaex7/8) 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 (Deltaex7/8) 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 (Deltaex7/8) mice show a progressive decline in visual acuity after 12 months of age.|Aging/*physiology[MESH]|Animals[MESH]|Color Vision/physiology[MESH]|Disease Models, Animal[MESH]|Disease Progression[MESH]|Electroretinography[MESH]|Gene Knock-In Techniques[MESH]|Homozygote[MESH]|Membrane Glycoproteins/*genetics/*physiology[MESH]|Mice[MESH]|Mice, Inbred C57BL[MESH]|Mice, Mutant Strains[MESH]|Molecular Chaperones/*genetics/*physiology[MESH]|Neuronal Ceroid-Lipofuscinoses/*genetics/*physiopathology[MESH]|Night Vision/physiology[MESH]|Retina/*physiopathology[MESH] |