Lysobisphosphatidic acid (LBPA) enrichment promotes cholesterol egress via exosomes in Niemann Pick type C1 deficient cells

•Phosphatidylglycerol (PG) incubation of NPC1 disease cells raises lysobisphosphatidic acid (LBPA) and reduces endolysosomal cholesterol.•Marked enrichment of LBPA species containing polyunsaturated acyl chains is observed following di-oleoyl PG treatment.•PG treatment/LBPA enrichment stimulates the secretion of exosomes, and these contain increased cholesterol compared to untreated cells.•Increasing LBPA reduces endolysosomal cholesterol, at least in part, by stimulating extracellular vesicle secretion.