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Iminosugar-Dihydroazulenes as Mutant L444P Glucocerebrosidase Enhancers.
- Source :
-
Chemistry & biodiversity [Chem Biodivers] 2024 Aug; Vol. 21 (8), pp. e202401104. Date of Electronic Publication: 2024 Jul 23. - Publication Year :
- 2024
-
Abstract
- A remarkable enhancer of human glucocerebrosidase enzyme (GCase) was identified among a set of dihydroazulene-tagged iminosugars. An unprecedented 3.9-fold increase in GCase activity was detected on fibroblasts bearing the homozygous L444P mutation, which is frequently associated with neuronopathic Gaucher forms, and which commonly results refractory to chaperone-induced refolding.<br /> (© 2024 The Author(s). Chemistry & Biodiversity published by Wiley-VHCA AG.)
- Subjects :
- Humans
Imino Sugars chemistry
Imino Sugars pharmacology
Imino Sugars chemical synthesis
Imino Sugars metabolism
Fibroblasts drug effects
Fibroblasts metabolism
Gaucher Disease genetics
Gaucher Disease drug therapy
Gaucher Disease metabolism
Molecular Structure
Glucosylceramidase metabolism
Glucosylceramidase genetics
Glucosylceramidase antagonists & inhibitors
Mutation
Subjects
Details
- Language :
- English
- ISSN :
- 1612-1880
- Volume :
- 21
- Issue :
- 8
- Database :
- MEDLINE
- Journal :
- Chemistry & biodiversity
- Publication Type :
- Academic Journal
- Accession number :
- 38847390
- Full Text :
- https://doi.org/10.1002/cbdv.202401104