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Neuroinflammation bei neuronalen Ceroid-Lipofuszinosen

Neuroinflammation in neuronal ceroid lipofuscinosis

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Zusammenfassung

Hintergrund

Netzhautdegeneration und Neuroinflammation sind meist frühe Merkmale verschiedener Subtypen von neuronalen Ceroid-Lipofuszinosen (NCL) bei Patienten und in genetischen Tiermodellen.

Fragestellung

Es soll ein Überblick über den aktuellen Forschungsstand zum Thema Neuroinflammation bei NCL gegeben werden.

Material und Methoden

Es erfolgen eine Betrachtung von relevanten Veröffentlichungen in PubMed sowie die Darstellung eigener Forschungsdaten.

Ergebnisse

Im zentralen Nervensystem (ZNS) und in der Netzhaut von NCL-Patienten und Tiermodellen werden Mikroglia und andere Gliazellen chronisch aktiviert und zeigen verschiedenste Dysfunktionen. Dies geht mit signifikanten Änderungen in ihrem Transkriptom und Proteom einher. Bei NCL findet sich ebenfalls eine Beteiligung der adaptiven Immunantwort, nachgewiesen durch das Einwandern von Autoantikörpern und aktivierten T‑Zellen.

Schlussfolgerungen

Das tiefere Verständnis der molekularen Abläufe, die zur Neuroinflammation beitragen und letztendlich zum Absterben von Neuronen führen, stellt eine wichtige Basis für die Entdeckung möglicher Biomarker und die Entwicklung von Immuntherapien bei der NCL dar.

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.

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Authors and Affiliations

  1. Lehrstuhl für Experimentelle Immunologie des Auges, Zentrum für Augenheilkunde, Medizinische Fakultät und Uniklinik Köln, Joseph-Stelzmann-Str. 9, 50931, Köln, Deutschland

    V. Behnke &  T. Langmann

  2. Zentrum für Molekulare Medizin, Köln, Deutschland

    T. Langmann

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Correspondence to T. Langmann.

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V. Behnke und T. Langmann geben an, dass kein Interessenkonflikt besteht. Für diesen Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgeführt. Die präsentierten Daten sind eine Rekapitulation bereits vorher publizierter Daten. Die in diesem Artikel modifiziert enthaltenen Bilder unterliegen der jeweiligen Creative-Commons-Lizenz.

Für diesen Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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Behnke, V., Langmann, T. Neuroinflammation bei neuronalen Ceroid-Lipofuszinosen. Ophthalmologe 118, 98–105 (2021). https://doi.org/10.1007/s00347-020-01301-4

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