Universität zu Köln

Volz, Cornelia, Mirza, Myriam, Langmann, Thomas and Jaegle, Herbert (2018). Further Characterization of the Predominant Inner Retinal Degeneration of Aging Cln3(Delta ex7/8) Knock-In Mice. S. 403 - 412. CHAM: SPRINGER INTERNATIONAL PUBLISHING AG. ISSN 2214-8019

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Abstract

Neuronal ceroid lipofuscinosis (NCL) is the most common group of neurogenetic storage diseases typically beginning in childhood. The juvenile form (JNCL), also known as Batten disease, is the most common form. Vision-related problems are often an early sign, appearing prior to motor and mental deficits. We have previously investigated disease progression with age in the Cln(3 Delta ex7/8) KI mouse model for JNCL and showed a decline of visual acuity and a predominant decline of the inner retinal function in mice, similar to human disease. The aim of this study was to further characterize this degeneration by means of flicker ERGs. For the scotopic flicker ERG, we found a significantly lower magnitude for Cln(3 Delta ex7/8) KI mice already at 6 months of age for low stimulus frequencies, while the difference declines with increasing frequency. Under photopic conditions there was no magnitude difference at 6 months, but a cumulative magnitude reduction with further aging. For both conditions the phases were similar for both groups. There was a similar magnitude reduction for the responses of both the slow and fast rod pathway in the 15 Hz experiments, and there were no differences in response phase. Low-frequency flicker responses seem to be sensitive to very early disease manifestations, and while the degeneration is associated with a reduction of predominating inner retinal responses in the scotopic flash ERG, this predominance seems not to be related to a selective involvement of the slow and fast rod pathways.

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:38-202088
DOI:	10.1007/978-3-319-75402-4_50
Title of Book:	Advances in Experimental Medicine and Biology
Series Name:	Adv.Exp.Med.Biol.
Volume:	1074
Page Range:	S. 403 - 412
Date:	2018
Publisher:	SPRINGER INTERNATIONAL PUBLISHING AG
Place of Publication:	CHAM
ISSN:	2214-8019
ISBN:	978-3-319-75402-4; 978-3-319-75401-7
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language ROD; PATHWAYS Multiple languages Cell Biology; Medicine, Research & Experimental; Ophthalmology Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/20208