Title : Treatment of neutral glycosphingolipid lysosomal storage diseases via inhibition of the ABC drug transporter, MDR1. Cyclosporin A can lower serum and liver globotriaosyl ceramide levels in the Fabry mouse model.

Pub. Date : 2006 May

PMID : 16724420






3 Functional Relationships(s)
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1 We have shown that the ABC transporter, multiple drug resistance protein 1 (MDR1, P-glycoprotein) translocates glucosyl ceramide from the cytosolic to the luminal Golgi surface for neutral, but not acidic, glycosphingolipid (GSL) synthesis. Glucosylceramides ATP-binding cassette, sub-family B (MDR/TAP), member 1B Mus musculus
2 We have shown that the ABC transporter, multiple drug resistance protein 1 (MDR1, P-glycoprotein) translocates glucosyl ceramide from the cytosolic to the luminal Golgi surface for neutral, but not acidic, glycosphingolipid (GSL) synthesis. Glucosylceramides ATP-binding cassette, sub-family B (MDR/TAP), member 1B Mus musculus
3 Here we show that the MDR1 inhibitor, cyclosporin A (CsA) can deplete Gaucher lymphoid cell lines of accumulated glucosyl ceramide and Fabry cell lines of globotriaosyl ceramide (Gb3), by preventing de novo synthesis. Glucosylceramides ATP-binding cassette, sub-family B (MDR/TAP), member 1B Mus musculus