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Accelerated transport and maturation of lysosomal α–galactosidase A in Fabry lymphoblasts by an enzyme inhibitor

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What this work says

Outgoing claims: the papers this work cites, builds on, or argues with.

inferredcites →https://doi.org/10.1006/abio.1997.2022openalex-snapshot
[14C]Ceramide Synthesis by Sphingolipid CeramideN-Deacylase: New Assay for Ceramidase Activity Detection
inferredcites →https://doi.org/10.1006/bbrc.1993.2659openalex-snapshot
Characterization of a Mutant α-Galactosidase Gene Product for the Late-Onset Cardiac Form of Fabry Disease
inferredcites →https://doi.org/10.1006/bbrc.1996.0486openalex-snapshot
Aggregation of the Inactive Form of Human α-Galactosidase in the Endoplasmic Reticulum
inferredcites →https://doi.org/10.1007/bf00207037openalex-snapshot
Point mutations in the upstream region of the ?-galactosidase A gene exon 6 in an atypical variant of Fabry disease
inferredcites →https://doi.org/10.1007/bf00210592openalex-snapshot
Intracellular processing and maturation of mutant gene products in hereditary ?-galactosidase deficiency (?-galactosidosis)
inferredcites →https://doi.org/10.1007/bf00484919openalex-snapshot
Residual activity of α-galactosidase A in Fabry's disease
inferredcites →https://doi.org/10.1016/0005-2760(95)00217-0openalex-snapshot
Induction of glucosylceramide synthase by synthase inhibitors and ceramide
inferredcites →https://doi.org/10.1016/0076-6879(74)31005-1openalex-snapshot
[2] Subcellular fractionation of rat liver
inferredcites →https://doi.org/10.1016/s0021-9258(18)61618-7openalex-snapshot
Synthesis and processing of alpha-galactosidase A in human fibroblasts. Evidence for different mutations in Fabry disease.
inferredcites →https://doi.org/10.1021/bi00323a042openalex-snapshot
Synthesis of lysogangliosides
inferredcites →https://doi.org/10.1021/bi00335a022openalex-snapshot
Reversible inhibitors of .beta.-glucosidase
inferredcites →https://doi.org/10.1021/jm00048a006openalex-snapshot
Nitrogen-in-the-Ring Pyranoses and Furanoses: Structural Basis of Inhibition of Mammalian Glycosidases
inferredcites →https://doi.org/10.1021/np960577nopenalex-snapshot
Homonojirimycin Isomers and Glycosides from Aglaonema treubii
inferredcites →https://doi.org/10.1023/a:1006915926732openalex-snapshot
α-Galactosidase transgenic mouse: Heterogeneous gene expression and posttranslational glycosylation in tissues
inferredcites →https://doi.org/10.1038/nm0598-610openalex-snapshot
Treatment of chronic hepadnavirus infection in a woodchuck animal model with an inhibitor of protein folding and trafficking
inferredcites →https://doi.org/10.1056/nejm196705252762101openalex-snapshot
Enzymatic Defect in Fabry's Disease
inferredcites →https://doi.org/10.1073/pnas.87.7.2725openalex-snapshot
Analysis of cytokine mRNA and DNA: detection and quantitation by competitive polymerase chain reaction.
inferredcites →https://doi.org/10.1146/annurev.cb.05.110189.001425openalex-snapshot
Protein Oligomerization in the Endoplasmic Reticulum

What points at this work

Incoming claims: where this work appears in other papers’ reference lists and arguments.

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Fabry Disease
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Mucopolysaccharidosis I
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GPCR Folding and Maturation
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inferred← citeshttps://doi.org/10.1007/s10545-009-1077-0openalex-snapshot
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inferred← citeshttps://doi.org/10.1007/s13730-020-00510-9openalex-snapshot
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inferred← citeshttps://doi.org/10.1007/s40259-025-00723-3openalex-snapshot
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inferred← citeshttps://doi.org/10.1007/s40265-019-01090-4openalex-snapshot
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inferred← citeshttps://doi.org/10.1016/b978-0-12-409547-2.02835-3openalex-snapshot
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inferred← citeshttps://doi.org/10.1016/b978-0-12-409547-2.14947-9openalex-snapshot
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inferred← citeshttps://doi.org/10.1016/b978-0-12-410529-4.00038-3openalex-snapshot
Fabry Disease
inferred← citeshttps://doi.org/10.1016/b978-0-12-449851-8.00035-8openalex-snapshot
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inferred← citeshttps://doi.org/10.1016/b978-0-12-802761-5.00027-4openalex-snapshot
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Fabry disease: α-galactosidase A deficiency
inferred← citeshttps://doi.org/10.1016/b978-0-12-816675-8.00008-7openalex-snapshot
Iminosugars
inferred← citeshttps://doi.org/10.1016/b978-0-12-816675-8.00010-5openalex-snapshot
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Structure-guided discovery of pharmacological chaperones targeting protein conformational and misfolding diseases
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Cellular high-throughput screening
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Glycan Arrays: Construction, Detection, and Analysis
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Yoshiyuki Suzuki
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Fabry disease: α-galactosidase A deficiency
inferred← citeshttps://doi.org/10.1016/b978-008045382-8.00125-8openalex-snapshot
Molecular Probes for Protein Glycosylation
inferred← citeshttps://doi.org/10.1016/b978-008045382-8.00649-3openalex-snapshot
Alkaloid Glycosidase Inhibitors
inferred← citeshttps://doi.org/10.1016/b978-012352551-2/50030-5openalex-snapshot
THE CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR (ABCC7)
inferred← citeshttps://doi.org/10.1016/b978-044451967-2/00046-5openalex-snapshot
Folding and Quality Control of Glycoproteins
inferred← citeshttps://doi.org/10.1016/b978-044451967-2/00100-8openalex-snapshot
Glycosidase Inhibitors: Structure, Activity, Synthesis, and Medical Relevance
inferred← citeshttps://doi.org/10.1016/bs.accb.2016.08.002openalex-snapshot
Carbohydrate-Processing Enzymes of the Lysosome
inferred← citeshttps://doi.org/10.1016/bs.pmbts.2022.03.003openalex-snapshot
Mutations in melanocortin-4 receptor: From fish to men
inferred← citeshttps://doi.org/10.1016/j.bbadis.2004.03.007openalex-snapshot
N-Octyl-β-valienamine up-regulates activity of F213I mutant β-glucosidase in cultured cells: a potential chemical chaperone therapy for Gaucher disease
inferred← citeshttps://doi.org/10.1016/j.bbadis.2004.07.001openalex-snapshot
Transgenic mouse expressing human mutant α-galactosidase A in an endogenous enzyme deficient background: a biochemical animal model for studying active-site specific chaperone therapy for Fabry disease
inferred← citeshttps://doi.org/10.1016/j.bbadis.2005.05.002openalex-snapshot
Lysosomal leukocyte β-d-glucuronidase during enzyme replacement therapy in Fabry disease
inferred← citeshttps://doi.org/10.1016/j.bbadis.2007.02.003openalex-snapshot
Enzyme enhancement activity of N-octyl-β-valienamine on β-glucosidase mutants associated with Gaucher disease
inferred← citeshttps://doi.org/10.1016/j.bbadis.2008.03.001openalex-snapshot
Rescue of mutant α-galactosidase A in the endoplasmic reticulum by 1-deoxygalactonojirimycin leads to trafficking to lysosomes
inferred← citeshttps://doi.org/10.1016/j.bbagen.2019.129437openalex-snapshot
Progress in the understanding and treatment of Fabry disease
inferred← citeshttps://doi.org/10.1016/j.bbalip.2013.10.015openalex-snapshot
Sphingolipids and lysosomal pathologies
inferred← citeshttps://doi.org/10.1016/j.bbamem.2006.05.027openalex-snapshot
Sphingolipid metabolism diseases
inferred← citeshttps://doi.org/10.1016/j.bbapap.2006.08.012openalex-snapshot
Small molecule pharmacological chaperones: From thermodynamic stabilization to pharmaceutical drugs
inferred← citeshttps://doi.org/10.1016/j.bbrc.2007.05.082openalex-snapshot
Screening for pharmacological chaperones in Fabry disease
inferred← citeshttps://doi.org/10.1016/j.bbrc.2007.06.048openalex-snapshot
4-Phenylbutyrate rescues trafficking incompetent mutant α-galactosidase A without restoring its functionality
inferred← citeshttps://doi.org/10.1016/j.bbrc.2011.10.038openalex-snapshot
Proteasome inhibitors improve the function of mutant lysosomal α-glucosidase in fibroblasts from Pompe disease patient carrying c.546G>T mutation
inferred← citeshttps://doi.org/10.1016/j.bcmd.2005.05.007openalex-snapshot
Miglustat (NB-DNJ) works as a chaperone for mutated acid β-glucosidase in cells transfected with several Gaucher disease mutations
inferred← citeshttps://doi.org/10.1016/j.bcmd.2008.11.002openalex-snapshot
Promising results of the chaperone effect caused by iminosugars and aminocyclitol derivatives on mutant glucocerebrosidases causing Gaucher disease
inferred← citeshttps://doi.org/10.1016/j.bcmd.2009.10.003openalex-snapshot
The pharmacological chaperone effect of N-octyl-β-valienamine on human mutant acid β-glucosidases
inferred← citeshttps://doi.org/10.1016/j.bcp.2026.118323openalex-snapshot
Pain in Fabry disease: do experimental models reveal novel therapeutic targets?
inferred← citeshttps://doi.org/10.1016/j.biochi.2015.03.018openalex-snapshot
Taming molecular flexibility to tackle rare diseases
inferred← citeshttps://doi.org/10.1016/j.biochi.2016.09.011openalex-snapshot
Alterations in membrane trafficking and pathophysiological implications in lysosomal storage disorders
inferred← citeshttps://doi.org/10.1016/j.bioorg.2024.107295openalex-snapshot
Multivalent pyrrolidines acting as pharmacological chaperones against Gaucher disease
inferred← citeshttps://doi.org/10.1016/j.biopha.2020.110779openalex-snapshot
Therapeutic advances in Fabry disease: The future awaits
inferred← citeshttps://doi.org/10.1016/j.bmc.2003.12.016openalex-snapshot
Convenient synthesis and evaluation of glycosidase inhibitory activity of α- and β-galactose-type valienamines, and some N-alkyl derivatives
inferred← citeshttps://doi.org/10.1016/j.bmc.2003.12.040openalex-snapshot
Design and synthesis of 2-acetamidomethyl derivatives of isofagomine as potential inhibitors of human lysosomal β-hexosaminidases
inferred← citeshttps://doi.org/10.1016/j.bmc.2006.05.080openalex-snapshot
Search for α-glucosidase inhibitors: New N-substituted valienamine and conduramine F-1 derivatives
inferred← citeshttps://doi.org/10.1016/j.bmc.2006.08.003openalex-snapshot
α-1-C-Octyl-1-deoxynojirimycin as a pharmacological chaperone for Gaucher disease
inferred← citeshttps://doi.org/10.1016/j.bmc.2008.06.026openalex-snapshot
In vitro inhibition of glycogen-degrading enzymes and glycosidases by six-membered sugar mimics and their evaluation in cell cultures
inferred← citeshttps://doi.org/10.1016/j.bmc.2013.05.056openalex-snapshot
Three dimensional structure of a bacterial α-l-fucosidase with a 5-membered iminocyclitol inhibitor
inferred← citeshttps://doi.org/10.1016/j.bmc.2013.06.022openalex-snapshot
Structure–activity relationship studies of Niemann-Pick type C1-like 1 (NPC1L1) ligands identified by screening assay monitoring pharmacological chaperone effect
inferred← citeshttps://doi.org/10.1016/j.bmc.2013.06.054openalex-snapshot
Rapid modifications of N-substitution in iminosugars: Development of new β-glucocerebrosidase inhibitors and pharmacological chaperones for Gaucher disease
inferred← citeshttps://doi.org/10.1016/j.bmc.2014.02.057openalex-snapshot
Docking and SAR studies of calystegines: Binding orientation and influence on pharmacological chaperone effects for Gaucher’s disease
inferred← citeshttps://doi.org/10.1016/j.bmcl.2010.11.112openalex-snapshot
The synthesis and biological evaluation of 1-C-alkyl-l-arabinoiminofuranoses, a novel class of α-glucosidase inhibitors
inferred← citeshttps://doi.org/10.1016/j.bmcl.2014.05.064openalex-snapshot
Structure–activity relationships of oxysterol-derived pharmacological chaperones for Niemann–Pick type C1 protein
inferred← citeshttps://doi.org/10.1016/j.bmcl.2017.05.086openalex-snapshot
A new type of pharmacological chaperone for GM1-gangliosidosis related human lysosomal β-galactosidase: N-Substituted 5-amino-1-hydroxymethyl-cyclopentanetriols
inferred← citeshttps://doi.org/10.1016/j.bmcl.2022.128571openalex-snapshot
Amyloidogenic immunoglobulin light chain kinetic stabilizers comprising a simple urea linker module reveal a novel binding sub-site
inferred← citeshttps://doi.org/10.1016/j.bmcl.2025.130426openalex-snapshot
In vitro comparison of the glycosidase inhibitory profile of isoiminosugars with their 1- and 5a-modified derivatives
inferred← citeshttps://doi.org/10.1016/j.bpc.2022.106915openalex-snapshot
A theoretical study on binding and stabilization of galactose and novel galactose analogues to the human α-galactosidase A variant causing Fabry disease
inferred← citeshttps://doi.org/10.1016/j.braindev.2004.01.005openalex-snapshot
Recent advances in the biochemistry and genetics of sphingolipidoses
inferred← citeshttps://doi.org/10.1016/j.braindev.2006.02.002openalex-snapshot
Fibroblast screening for chaperone therapy in β-galactosidosis
inferred← citeshttps://doi.org/10.1016/j.braindev.2006.08.014openalex-snapshot
Motor and reflex testing in GM1-gangliosidosis model mice
inferred← citeshttps://doi.org/10.1016/j.braindev.2009.12.005openalex-snapshot
The effect of N-octyl-β-valienamine on β-glucosidase activity in tissues of normal mice
inferred← citeshttps://doi.org/10.1016/j.braindev.2011.01.007openalex-snapshot
Molecular basis of neurogenetic diseases
inferred← citeshttps://doi.org/10.1016/j.braindev.2012.12.002openalex-snapshot
Chaperone therapy update: Fabry disease, GM1-gangliosidosis and Gaucher disease
inferred← citeshttps://doi.org/10.1016/j.braindev.2020.06.015openalex-snapshot
Chaperone therapy for molecular pathology in lysosomal diseases
inferred← citeshttps://doi.org/10.1016/j.braindev.2023.02.004openalex-snapshot
Chaperone therapy for lysosomal and non-lysosomal protein misfolding diseases
inferred← citeshttps://doi.org/10.1016/j.braindev.2024.10.006openalex-snapshot
Chaperone therapy: Stabilization and enhancement of endogenous and exogenous lysosomal enzymes
inferred← citeshttps://doi.org/10.1016/j.brainres.2006.11.039openalex-snapshot
Cerebrospinal fluid biomarkers showing neurodegeneration in dogs with GM1 gangliosidosis: Possible use for assessment of a therapeutic regimen
inferred← citeshttps://doi.org/10.1016/j.carres.2007.03.015openalex-snapshot
Synthesis of 4-O-glycosylated 1-deoxynojirimycin derivatives as disaccharide mimics-based inhibitors of human β-glucocerebrosidase
inferred← citeshttps://doi.org/10.1016/j.carres.2008.07.013openalex-snapshot
Synthesis of iminoalditol analogues of galactofuranosides and their activities against glycosidases
inferred← citeshttps://doi.org/10.1016/j.carres.2010.01.021openalex-snapshot
Synthesis of N-substituted iminosugar derivatives and their immunosuppressive activities
inferred← citeshttps://doi.org/10.1016/j.carres.2012.03.015openalex-snapshot
I2-mediated carbamate annulation: scope and application in the synthesis of azasugars
inferred← citeshttps://doi.org/10.1016/j.carres.2012.12.010openalex-snapshot
Concise syntheses of potent chaperone drug candidates, N-octyl-4-epi-β-valinenamine (NOEV) and its 6-deoxy derivative, from (+)-proto-quercitol
inferred← citeshttps://doi.org/10.1016/j.carres.2016.03.007openalex-snapshot
Investigation of original multivalent iminosugars as pharmacological chaperones for the treatment of Gaucher disease
inferred← citeshttps://doi.org/10.1016/j.carres.2016.03.010openalex-snapshot
Molecular basis for the affinity and specificity in the binding of five-membered iminocyclitols with glycosidases: an experimental and theoretical synergy
inferred← citeshttps://doi.org/10.1016/j.carres.2016.09.003openalex-snapshot
One pot oxidative dehydration - oxidation of polyhydroxyhexanal oxime to polyhydroxy oxohexanenitrile: A versatile methodology for the facile access of azasugar alkaloids
inferred← citeshttps://doi.org/10.1016/j.carres.2017.03.009openalex-snapshot
N-Substituted 5-amino-1-hydroxymethyl-cyclopentanetriols: A new family of activity promotors for a G M1 -gangliosidosis related human lysosomal β-galactosidase mutant
inferred← citeshttps://doi.org/10.1016/j.cbpa.2004.04.001openalex-snapshot
Recent applications of olefin metathesis to combinatorial chemistry
inferred← citeshttps://doi.org/10.1016/j.cbpa.2008.05.010openalex-snapshot
Mechanistic insights into glycosidase chemistry
inferred← citeshttps://doi.org/10.1016/j.cca.2018.02.021openalex-snapshot
Functional and pharmacological evaluation of novel GLA variants in Fabry disease identifies six (two de novo ) causative mutations and two amenable variants to the chaperone DGJ
inferred← citeshttps://doi.org/10.1016/j.cca.2024.119843openalex-snapshot
Fabry disease Enzyme Enhancement on migalastat Study: FEES
inferred← citeshttps://doi.org/10.1016/j.ceb.2010.11.002openalex-snapshot
Chemical and/or biological therapeutic strategies to ameliorate protein misfolding diseases
inferred← citeshttps://doi.org/10.1016/j.cell.2008.06.037openalex-snapshot
Chemical and Biological Approaches Synergize to Ameliorate Protein-Folding Diseases
inferred← citeshttps://doi.org/10.1016/j.celrep.2022.111096openalex-snapshot
Pulse labeling reveals the tail end of protein folding by proteome profiling
inferred← citeshttps://doi.org/10.1016/j.ces.2021.116602openalex-snapshot
Multi-reaction kinetic modeling for the peroxidase–aldolase cascade synthesis of a D-fagomine precursor
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