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The origin of fluorescence in the neuronal ceroid lipofuscinoses (Batten disease) and neuron cultures from affected sheep for studies of neurodegeneration by David N Palmer; Manfred J Oswald; Valerie J Westlake; Graham W Kay is a Medicine article available to read on EtoBox.

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Lipofuscin and ceroid are usually held responsible for impaired cellular performance, via oxidative damage and the irreversible accumulation of fluorescent products of lipid peroxidation. The neuronal ceroid lipofuscinoses (NCLs, Batten disease) are inherited neurodegenerative diseases characterized by intracellular accumulation of fluorescent lipofuscin-like bodies. However these bodies are lysosomes packed with a particular protein, subunit c of mitochondrial ATP synthase; not the result of oxidative damage. No individual storage body component was fluorescent nor were solutions of total storage bodies. UV-vis spectra confirmed the lack of a fluorophor. Crystals of non-fluorescent albumin and reconstituted storage bodies were fluorescent in glycerol suspensions. This fluorescence is probably caused by interference of light reflected from the protein array, as is often observed in protein crystals. Other lipofuscins may be secondary lysosomes with a high protein content and the source of fluorescence the same. The neurodegeneration associated with lipofuscin accumulation may be caused by that accumulation, or may be a separate manifestation of aging. Neuronal cell cultures offer a

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Author

David N Palmer; Manfred J Oswald; Valerie J Westlake; Graham W Kay

Publisher

Elsevier Science; Elsevier ; Elsevier BV (ISSN 0167-4943)

Published

2002

Language

EN

Field

Medicine (Health Sciences)