Without prosaposin, glycosphingolipids accumulate in the lysosome, which form lipofuscin granules. The iron in these granules produces reactive oxygen species, which oxidize lipids. Peroxidized lipids ...

In neurons, lipofuscin is commonly observed in lysosomal storage diseases caused by mutations in genes encoding proteins essential for lysosomal degradation and recycling of cellular macromolecules.

Scientists at the Gladstone Institutes and the University of California, San Francisco (UCSF), identified a common mechanism in two forms of neurodegeneration that affect young adults or the elderly.