Iminosugar‐Dihydroazulenes as Mutant L444P Glucocerebrosidase Enhancers
Copyright policy )Iminosugar‐Dihydroazulenes as Mutant L444P Glucocerebrosidase Enhancers
AbstractA 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.
- University of Copenhagen Denmark
- Meyer Children's Hospital Italy
- University of Copenhagen Denmark
- New York University Italy
- University of Florence Italy
Enzyme modulators, Gaucher Disease, Molecular Structure, Click chemistry, Neuronopathic disorder, Gaucher disease, Fibroblasts, Photochromism, Imino Sugars, Mutation, Humans, Glucosylceramidase, Gaucher disease; Enzyme modulators; Neuronopathic disorder; Photochromism; Click chemistry
Enzyme modulators, Gaucher Disease, Molecular Structure, Click chemistry, Neuronopathic disorder, Gaucher disease, Fibroblasts, Photochromism, Imino Sugars, Mutation, Humans, Glucosylceramidase, Gaucher disease; Enzyme modulators; Neuronopathic disorder; Photochromism; Click chemistry
selected citations These citations are derived from selected sources.This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).3 popularity This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.Top 10% influence This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).Average impulse This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.Average
Citations provided by BIP!Popularity provided by BIP!