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Article

Iminosugar-Dihydroazulenes as Mutant L444P Glucocerebrosidase Enhancers.

Chemistry & biodiversity - 1 Aug 2024

Davighi Maria Giulia, Clemente Francesca, Andreasen Emilie Sperling, Nielsen Mogens Brøndsted, Matassini Camilla, Goti Andrea, Morrone Amelia, Paoli Paolo, Cardona Francesca, Cacciarini Martina

Abstract excerpt

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.

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Identifiers

PMID: 38847390
DOI: 10.1002/cbdv.202401104

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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