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Der Ophthalmologe : Zeitschrift der Deutschen Ophthalmologischen Gesellschaft · 2021

[Neuroinflammation in neuronal ceroid lipofuscinosis].

СтатьяОбзорНазвание и аннотация: Перевод в очереди
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Behnke V, Langmann T

Аннотация

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.

Предметные рубрики MeSH

ИП Урсолов Владлен Сергеевич

ИНН 500118482352 · ОГРНИП 314500117700049

Реквизиты и контакты