Journal article

Effect of lysosomal storage on bis(monoacylglycero)phosphate

Peter J Meikle, Stephen Duplock, David Blacklock, Phillip D Whitfield, Gemma Macintosh, John J Hopwood, Maria Fuller

Biochemical Journal | PORTLAND PRESS LTD | Published : 2008

Abstract

BMP [bis(monoacylglycero)phosphate] is an acidic phospholipid and a structural isomer of PG (phosphatidylglycerol), consisting of lysophosphatidylglycerol with an additional fatty acid esterified to the glycerol head group. It is thought to be synthesized from PG in the endosomal/lysosomal compartment and is found primarily in multivesicular bodies within the same compartment. In the present study, we investigated the effect of lysosomal storage on BMP in cultured fibroblasts from patients with eight different LSDs (lysosomal storage disorders) and plasma samples from patients with one of 20 LSDs. Using ESI-MS/MS (electrospray ionization tandem MS), we were able to demonstrate either elevati..

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