Isofagomine- and 2,5-anhydro-2,5-imino-D-glucitol-based glucocerebrosidase pharmacological chaperones for Gaucher disease intervention.
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Molecular basis of reduced glucosylceramidase activity in the most common Gaucher disease mutant, N370SChemical and biological approaches for adapting proteostasis to ameliorate protein misfolding and aggregation diseases: progress and prognosisBinding of 3,4,5,6-Tetrahydroxyazepanes to the Acid-β-glucosidase Active Site: Implications for Pharmacological Chaperone Design for Gaucher DiseasePartial restoration of mutant enzyme homeostasis in three distinct lysosomal storage disease cell lines by altering calcium homeostasisThree classes of glucocerebrosidase inhibitors identified by quantitative high-throughput screening are chaperone leads for Gaucher diseaseIdentification and characterization of ambroxol as an enzyme enhancement agent for Gaucher diseaseHigh-throughput screening for inhibitors of Mycobacterium tuberculosis H37Rv.Fluorous iminoalditols: a new family of glycosidase inhibitors and pharmacological chaperones.Using pharmacological chaperones to restore proteostasisBicyclic (galacto)nojirimycin analogues as glycosidase inhibitors: effect of structural modifications in their pharmacological chaperone potential towards β-glucocerebrosidase.The pharmacological chaperone isofagomine increases the activity of the Gaucher disease L444P mutant form of beta-glucosidase.Multi-system disorders of glycosphingolipid and ganglioside metabolismDiscovery, structure-activity relationship, and biological evaluation of noninhibitory small molecule chaperones of glucocerebrosidaseThe lipophilic bullet hits the targets: medicinal chemistry of adamantane derivativesRemodeling the proteostasis network to rescue glucocerebrosidase variants by inhibiting ER-associated degradation and enhancing ER folding.Chemical and biological approaches synergize to ameliorate protein-folding diseases.Evaluation of quinazoline analogues as glucocerebrosidase inhibitors with chaperone activityHistone deacetylase inhibitors prevent the degradation and restore the activity of glucocerebrosidase in Gaucher diseaseNon-iminosugar glucocerebrosidase small molecule chaperones.N4-phenyl modifications of N2-(2-hydroxyl)ethyl-6-(pyrrolidin-1-yl)-1,3,5-triazine-2,4-diamines enhance glucocerebrosidase inhibition by small molecules with potential as chemical chaperones for Gaucher diseaseThe unfolded protein response: a pathway that links insulin demand with beta-cell failure and diabetesChemical and pharmacological chaperones as new therapeutic agents.Improved management of lysosomal glucosylceramide levels in a mouse model of type 1 Gaucher disease using enzyme and substrate reduction therapyPharmacologic chaperoning as a strategy to treat Gaucher disease.Glycan antagonists and inhibitors: a fount for drug discovery.Synthesis and characterization of 2-substituted bornane pharmacophores for novel cannabinergic ligandsReview of the use of idursulfase in the treatment of mucopolysaccharidosis II.Glycosphingolipids--nature, function, and pharmacological modulation.Pharmacological small molecules for the treatment of lysosomal storage disorders.Pharmacological chaperone therapy for Gaucher disease: a patent review.Metathesis access to monocyclic iminocyclitol-based therapeutic agents.Gelsolin amyloidosis: genetics, biochemistry, pathology and possible strategies for therapeutic intervention.Glucocerebrosidase inhibitors for the treatment of Gaucher disease.sp2-Iminosugar O-, S-, and N-glycosides as conformational mimics of α-linked disaccharides; implications for glycosidase inhibition.Pharmacological Chaperone Therapy: Preclinical Development, Clinical Translation, and Prospects for the Treatment of Lysosomal Storage Disorders.Expanding role of molecular chaperones in regulating α-synuclein misfolding; implications in Parkinson's disease.1-Deoxynojirimycins with dansyl capped N-substituents as probes for Morbus Gaucher affected cell lines.Isofagomine induced stabilization of glucocerebrosidase.Catalyst-free multicomponent synthesis of novel adamantyl-containing tetrahydropyrimidine carboxylates.Human Acid β-Glucosidase Inhibition by Carbohydrate Derived Iminosugars: Towards New Pharmacological Chaperones for Gaucher Disease.
P2860
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P2860
Isofagomine- and 2,5-anhydro-2,5-imino-D-glucitol-based glucocerebrosidase pharmacological chaperones for Gaucher disease intervention.
description
2007 nî lūn-bûn
@nan
2007年の論文
@ja
2007年論文
@yue
2007年論文
@zh-hant
2007年論文
@zh-hk
2007年論文
@zh-mo
2007年論文
@zh-tw
2007年论文
@wuu
2007年论文
@zh
2007年论文
@zh-cn
name
Isofagomine- and 2,5-anhydro-2 ...... Gaucher disease intervention.
@en
type
label
Isofagomine- and 2,5-anhydro-2 ...... Gaucher disease intervention.
@en
prefLabel
Isofagomine- and 2,5-anhydro-2 ...... Gaucher disease intervention.
@en
P2093
P2860
P356
P1476
Isofagomine- and 2,5-anhydro-2 ...... r Gaucher disease intervention
@en
P2093
Anu R Sawkar
Chi-Huey Wong
Zhanqian Yu
P2860
P304
P356
10.1021/JM060677I
P407
P577
2007-01-01T00:00:00Z