Turnover of Docosahexaenoic Acid in Bis(Monoacylglycero)Phosphate Induced in Niemann-Pick Fibroblasts by Incubation in the Presence of Excess Fatty Acid
The lysosomal phospholipid, bis(monoacylglycero)phosphate1 (BMP), accumulates selectively in both inherited2–4 and acquired5-6 disorders of lysosomal lipid catabolism. In those forms of Niemann-Pick disease with a profound deficiency of lysosomal sphingomyelinase, there is also an equally profound deficiency of phospholipase C7. Although sphingomyelin and phospholipids are substrates for the defective enzyme, there may be greater relative increase of BMP than of sphingomyelin and both sphingomyelin and BMP accumulate to a much greater degree than the other phospholipids. Lack of accumulation of phospholipids has been explained by the availability of other pathways excusive of phospholipase C for catabolism that are assumed to be unavailable to either sphingomyelin or BMP7.
KeywordsLinoleic Acid Neutral Lipid Docosahexaenoic Acid Exogenous Fatty Acid Perhexiline Maleate
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