1
|
A specific and potent inhibitor of glucosylceramide synthase for substrate inhibition therapy of Gaucher disease.
|
Mol Genet Metab
|
2007
|
1.61
|
2
|
Improved management of lysosomal glucosylceramide levels in a mouse model of type 1 Gaucher disease using enzyme and substrate reduction therapy.
|
J Inherit Metab Dis
|
2010
|
1.10
|
3
|
Substrate reduction augments the efficacy of enzyme therapy in a mouse model of Fabry disease.
|
PLoS One
|
2010
|
1.04
|
4
|
Iminosugar-based inhibitors of glucosylceramide synthase increase brain glycosphingolipids and survival in a mouse model of Sandhoff disease.
|
PLoS One
|
2011
|
0.92
|
5
|
Iminosugar-based inhibitors of glucosylceramide synthase prolong survival but paradoxically increase brain glucosylceramide levels in Niemann-Pick C mice.
|
Mol Genet Metab
|
2012
|
0.89
|
6
|
A glucosylceramide synthase inhibitor protects rats against the cytotoxic effects of shiga toxin 2.
|
Pediatr Res
|
2011
|
0.82
|
7
|
The chemosensitizing activity of inhibitors of glucosylceramide synthase is mediated primarily through modulation of P-gp function.
|
Int J Oncol
|
2010
|
0.80
|