Amphiphilic 1-deoxynojirimycin derivatives through click strategies for chemical chaperoning in N370S Gaucher cells.
In Gaucher disease (GD), mutant β-glucocerebrosidases (β-GCase) that are misfolded are recognized by the quality control machinery of the endoplasmic reticulum (ER) and degraded proteolytically. Hydrophobic iminosugars can be used as pharmacological chaperones to provide an improvement in the foldin...
Κύριοι συγγραφείς: | Diot, J, Garcia Moreno, I, Twigg, G, Ortiz Mellet, C, Haupt, K, Butters, T, Kovensky, J, Gouin, S |
---|---|
Μορφή: | Journal article |
Γλώσσα: | English |
Έκδοση: |
2011
|
Παρόμοια τεκμήρια
Παρόμοια τεκμήρια
-
Synthesis of N-substituted ε-hexonolactams as pharmacological chaperones for the treatment of N370S mutant Gaucher disease.
ανά: Wang, G, κ.ά.
Έκδοση: (2012) -
Crystal structures of complexes of N-butyl- and N-nonyl-deoxynojirimycin bound to acid beta-glucosidase: insights into the mechanism of chemical chaperone action in Gaucher disease.
ανά: Brumshtein, B, κ.ά.
Έκδοση: (2007) -
Rational design and synthesis of highly potent pharmacological chaperones for treatment of N370S mutant Gaucher disease.
ανά: Wang, G, κ.ά.
Έκδοση: (2009) -
Potent aminocyclitol glucocerebrosidase inhibitors are subnanomolar pharmacological chaperones for treating gaucher disease.
ανά: Trapero, A, κ.ά.
Έκδοση: (2012) -
Gaucher disease.
ανά: Butters, T
Έκδοση: (2007)