Progressive Retinal Degeneration and Glial Activation in the CLN6nclf Mouse Model of Neuronal Ceroid Lipofuscinosis: A Beneficial Effect of DHA and Curcumin Supplementation

URN to cite this document:: urn:nbn:de:bvb:355-epub-621673
DOI to cite this document:: 10.5283/epub.62167

Volz, Cornelia ; Mirza, Myriam ; Karlstetter, Marcus ; Langiu, Monica ; Somogyi, Aleksandra ; Ruonala, Mika O. ; Tamm, Ernst R.

; Jägle, Herbert ; Langmann, Thomas

License: Creative Commons Attribution 4.0
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Date of publication of this fulltext: 19 Dec 2024 08:34

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Abstract

Neuronal ceroid lipofuscinosis (NCL) is a group of neurodegenerative lysosomal storage disorders characterized by vision loss, mental and motor deficits, and spontaneous seizures. Neuropathological analyses of autopsy material from NCL patients and animal models revealed brain atrophy closely associated with glial activity. Earlier reports also noticed loss of retinal cells and reactive gliosis ...

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