Universität zu Köln

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

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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
Creators:	Creators Email ORCID ORCID Put Code Volz, Cornelia UNSPECIFIED UNSPECIFIED UNSPECIFIED Mirza, Myriam UNSPECIFIED UNSPECIFIED UNSPECIFIED Langmann, Thomas UNSPECIFIED UNSPECIFIED UNSPECIFIED Jaegle, Herbert UNSPECIFIED UNSPECIFIED UNSPECIFIED
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
Uncontrolled Keywords:	Keywords Language MOUSE MODEL; MUTATION; DISEASE; JNCL Multiple languages Biology; Medicine, Research & Experimental; Ophthalmology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/44899