Programm                 "Degeneration und Regeneration– Grundlagen, Diagnostik und Therapie"


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Abstract
Abstract

Evaluation of the Deafblind LEW/Ztm-ci2 Rat as a Model of Non-isolated Photoreceptor Degeneration

Gockeln R.1, Kamino K.2, Wedekind D.3, Chwalisz W.3, Winter R.1, Hedrich H.-J.3 
1Vision Science Laboratory, Department of Ophthalmology, 2Institute for Cell & Molecular Pathology, 3Institute for Laboratory Animal Science, Hannover Medical School

Purpose: Circling2 (ci2) is an autosomal recessive mutation that displays hyperactivity, head-tossing, circling behaviour, a complete sensorineural hearing loss and vestibular defects in LEW rats. In view of the similarities between the phenotypes of the ci2 rat mutant and the shaker-waltzer deafness mutants, the present study evaluated the retinal morphology and function of the ci2 rat mutants.
Method: Electroretinograms (ERGs) were obtaind from 12 homozygous LEW/Ztm-ci2 rats, and 12 heterozygous littermates. Ten age- and sex-matched LEW/Ztm rats served as controls. The same individuals were tested every two weeks, beginning at postnatal day (P)30. The ERG protocols included flash and flicker stimuli under scotopic and photopic conditions. Retinal morphology was analyzed in rats of comparable age.
Results: Light-evoked retinal responses of ci2 mutants demonstrated initial (P30) attenuation of scotopic and photopic a- and b-waves as compared to the controls. In both, homozygous and heterozygous, rat mutants at postnatal week (PW)14 the scotopic electroretinogram was significant decreased


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