Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Selective action of the iminosugar isofagomine, a pharmacological chaperone for mutant forms of acid-beta-glucosidase.

Biochemical pharmacology - 1 May 2007

Steet Richard, Chung Stephen, Lee Wang-Sik, Pine Corey W, Do Hung, Kornfeld Stuart

Abstract excerpt

Gaucher disease is a lysosomal glycolipid storage disorder characterized by defects in acid-beta-glucosidase (GlcCerase), the enzyme responsible for the catabolism of glucosylceramide. We recently demonstrated that isofagomine (IFG), an iminosugar that binds to the active site of GlcCerase, enhances the folding, transport and activity of the N370S mutant form of GlcCerase. In this study we compared the effects of...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to 1-Deoxynojirimycin
Share to alpha-Glucosidases

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Gaucher Disease

Share to Glucosylceramidase

Share to Glucosyltransferases

Share to Glycoside Hydrolases

Share to Oligosaccharides

Share to Piperidines

Source

Open in PubMed Open full text

Identifiers

PMID: 17217920
DOI: 10.1016/j.bcp.2006.12.015
PMCID: PMC1892903

Provenance

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.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Selective action of the iminosugar isofagomine, a pharmacological chaperone for mutant forms of acid-beta-glucosidase.PMID 17217920Read ↗

The iminosugar isofagomine increases the activity of N370S mutant acid beta-glucosidase in Gaucher fibroblasts by several mechanisms.Semantic proximity Read ↗

The pharmacological chaperone isofagomine increases the activity of the Gaucher disease L444P mutant form of beta-glucosidase.Semantic proximity Read ↗

Ex Vivo and in Vivo Effects of Isofagomine on Acid β-Glucosidase Variants and Substrate Levels in Gaucher DiseaseSemantic proximity Read ↗

Isofagomine- and 2,5-anhydro-2,5-imino-D-glucitol-based glucocerebrosidase pharmacological chaperones for Gaucher disease intervention.Semantic proximity Read ↗

Isofagomine induced stabilization of glucocerebrosidase.Semantic proximity Read ↗

Identification of pharmacological chaperones for Gaucher disease and characterization of their effects on beta-glucocerebrosidase by hydrogen/deuterium exchange mass spectrometry.Semantic proximity Read ↗

Rational Design and Synthesis of Highly Potent β‐Glucocerebrosidase InhibitorsSemantic proximity Read ↗

Glucosylceramide mimics: highly potent GCase inhibitors and selective pharmacological chaperones for mutations associated with types 1 and 2 Gaucher disease.Semantic proximity Read ↗

Miglustat (NB-DNJ) works as a chaperone for mutated acid beta-glucosidase in cells transfected with several Gaucher disease mutations.Semantic proximity Read ↗

Pharmacological chaperones for human α- <i>N</i> -acetylgalactosaminidaseSemantic proximity Read ↗

Probing the Inhibitor versus Chaperone Properties of sp²-Iminosugars towards Human β-Glucocerebrosidase: A Picomolar Chaperone for Gaucher Disease.Semantic proximity Read ↗

New insights into the pharmacological chaperone activity of c2-substituted glucoimidazoles for the treatment of Gaucher disease.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.