Universität zu Köln

Behnke, V. and Langmann, T. (2021). Neuroinflammation in neuronal ceroid lipofuscinosis. Ophthalmologe, 118 (2). S. 98 - 106. HEIDELBERG: SPRINGER HEIDELBERG. ISSN 1433-0423

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Abstract

Background Retinal degeneration and neuroinflammation are often early hallmarks of different subtypes of neuronal ceroid lipofuscinosis (NCL) in patients and genetic animal models. Objective This article gives a summary of recently published research articles and novel concepts in the field of NCL-related neuroinflammation. Material and methods A search was carried out in PubMed for relevant publications and the results as well as own NCL-related research are discussed. Results Microglia and other glial cells are chronically activated and show various dysfunctions in the central nervous system (CNS) and retina of NCL patients and animal models. This is accompanied by significant changes in the transcriptome and proteome. In NCL there is also involvement of the adaptive immune response, as demonstrated by the influx of autoantibodies and activated T cells. Conclusion A deeper understanding of the molecular processes that contribute to neuroinflammation and ultimately lead to neuronal cell death is an important basis for the discovery of possible biomarkers and the development of immunotherapies in NCL.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Behnke, V. UNSPECIFIED UNSPECIFIED UNSPECIFIED Langmann, T. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-573393
DOI:	10.1007/s00347-020-01301-4
Journal or Publication Title:	Ophthalmologe
Volume:	118
Number:	2
Page Range:	S. 98 - 106
Date:	2021
Publisher:	SPRINGER HEIDELBERG
Place of Publication:	HEIDELBERG
ISSN:	1433-0423
Language:	German
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language MOUSE MODEL; ANTIOXIDANT TREATMENT; MICE DEFICIENT; BRAIN; NEURODEGENERATION; AUTOANTIBODY; SYSTEM Multiple languages Ophthalmology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/57339