Article
Ellagic acid and its metabolites urolithins A/B ameliorate most common disease phenotypes in cellular and mouse models for lysosomal storage disorders by enhancing extracellular vesicle secretion.
Neurobiology of disease - 15 Jun 2023
Soto-Huelin Beatriz, Babiy Bohdan, Pastor Oscar, Díaz-García Mario, Toledano-Zaragoza Ana, Frutos María Dolores, Espín Juan Carlos, Tomás-Barberán Francisco A, Busto Rebeca, Ledesma María Dolores
Abstract excerpt
Niemann Pick diseases types A (NPDA) and C (NPDC) are lysosomal storage disorders (LSDs) leading to cognitive impairment, neurodegeneration, and early death. NPDA and NPDC have different genetic origins, being caused by mutations in the acid sphingomyelinase (ASM) or the cholesterol transport protein NPC1, respectively. However, they share a common pathological hallmark in the accumulation of lipids in the...
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