Selective reduction of bis(monoacylglycero)phosphate ameliorates the storage burden in a THP-1 macrophage model of Gaucher disease

Selective reduction of bis(monoacylglycero)phosphate ameliorates the storage burden in a THP-1 macrophage model of Gaucher disease

Bis(monoacylglycero)phosphate (BMP) assists lysosomal function by facilitating interaction of hydrolases and activator proteins with sphingolipid substrates. Impaired lysosomal degradation of the sphingolipid glucosylceramide (GC) occurs in Gaucher disease due to an inherited deficiency of acid β-gl...

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Bibliographic Details
Main Authors: Leanne K. Hein, Stephen Duplock, Maria Fuller
Format: Article
Language:English
Published: Elsevier 2013-06-01
Series:Journal of Lipid Research
Subjects:
detergent-resistant membranes
fatty acids
glucosylceramide
lysosomal disease
lysosomal dysfunction
lysosomal storage disorder
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520357242

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http://www.sciencedirect.com/science/article/pii/S0022227520357242

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