2024-03-28T09:39:56Z
http://repoint.unil.ch/oaiprovider/
oai:serval.unil.ch:BIB_3D4176C72BFD
2024-03-23T02:14:33Z
serval:BIB_3D4176C72BFD
(5aR)-5a-C-Pentyl-4-epi-isofagomine: A powerful inhibitor of lysosomal β-galactosidase and a remarkable chaperone for mutations associated with GM1-gangliosidosis and Morquio disease type B.
10.1016/j.ejmech.2016.09.095
27750150
000396804600016
Front
S.
author
Biela-Banaś
A.
author
Burda
P.
author
Ballhausen
D.
author
Higaki
K.
author
Caciotti
A.
author
Morrone
A.
author
Charollais-Thoenig
J.
author
Gallienne
E.
author
Demotz
S.
author
Martin
O.R.
author
article
2017-04-27
2017-04-27
European journal of medicinal chemistry
1768-3254
0223-5234
journal
126
160-170
Drug Design
Enzyme Inhibitors/chemical synthesis
Enzyme Inhibitors/chemistry
Enzyme Inhibitors/pharmacology
Fibroblasts/drug effects
Gangliosidosis, GM1/enzymology
Gangliosidosis, GM1/genetics
Gangliosidosis, GM1/pathology
Hot Temperature
Humans
Hydrogen-Ion Concentration
Imino Pyranoses/chemical synthesis
Imino Pyranoses/chemistry
Imino Pyranoses/pharmacology
Lysosomes/enzymology
Mucopolysaccharidosis IV/enzymology
Mucopolysaccharidosis IV/genetics
Mucopolysaccharidosis IV/pathology
Mutation
Protein Denaturation
beta-Galactosidase/antagonists & inhibitors
beta-Galactosidase/chemistry
beta-Galactosidase/genetics
beta-Galactosidase/metabolism
eng
60_published
peer-reviewed
Publication types: Journal Article
Publication Status: ppublish