Potent chemical chaperone compounds for GM1-gangliosidosis: N-substituted (+)-conduramine F-4 derivatives

Structural Basis of Pharmacological Chaperoning for Human β-Galactosidase

(2014) Hironori Suzuki et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Pharmacological Chaperones as Therapeutics for Lysosomal Storage Diseases

(2013) Robert E. Boyd et al.   JOURNAL OF MEDICINAL CHEMISTRY

A Bicyclic 1-Deoxygalactonojirimycin Derivative as a Novel Pharmacological Chaperone for GM1 Gangliosidosis

(2013) Tomoko Takai et al.   MOLECULAR THERAPY

Concise syntheses of potent chaperone drug candidates, N-octyl-4-epi-β-valinenamine (NOEV) and its 6-deoxy derivative, from (+)-proto-quercitol

(2012) Shinichi Kuno et al.   CARBOHYDRATE RESEARCH

Tuning glycosidase inhibition through aglycone interactions: pharmacological chaperones for Fabry disease and GM1 gangliosidosis

(2012) M. Aguilar-Moncayo et al.   CHEMICAL COMMUNICATIONS

Discovery, Structure–Activity Relationship, and Biological Evaluation of Noninhibitory Small Molecule Chaperones of Glucocerebrosidase

(2012) Samarjit Patnaik et al.   JOURNAL OF MEDICINAL CHEMISTRY

Evaluation of N-nonyl-deoxygalactonojirimycin as a pharmacological chaperone for human GM1 gangliosidosis leads to identification of a feline model suitable for testing enzyme enhancement therapy

(2012) Brigitte A. Rigat et al.   MOLECULAR GENETICS AND METABOLISM

Pharmacological Enhancement of α-Glucosidase by the Allosteric Chaperone N-acetylcysteine

(2012) Caterina Porto et al.   MOLECULAR THERAPY

Determining the Role of the Aromatic Ring ofN-Arylmethylent-conduramine F-1 in their Interactions with α-Glucosidases by Saturation Transfer Difference NMR Spectroscopy Experiments

(2012) Antonio Hernández Daranas et al.   ChemistryOpen

Identification and Characterization of Pharmacological Chaperones to Correct Enzyme Deficiencies in Lysosomal Storage Disorders

(2011) Kenneth J. Valenzano et al.   ASSAY AND DRUG DEVELOPMENT TECHNOLOGIES

1-Deoxy-d-galactonojirimycins with dansyl capped N-substituents as β-galactosidase inhibitors and potential probes for GM1 gangliosidosis affected cell lines

(2011) Richard F.G. Fröhlich et al.   CARBOHYDRATE RESEARCH

Chemical chaperone therapy: chaperone effect on mutant enzyme and cellular pathophysiology in β-galactosidase deficiency

(2011) Katsumi Higaki et al.   HUMAN MUTATION

DLHex-DGJ, a novel derivative of 1-deoxygalactonojirimycin with pharmacological chaperone activity in human GM1-gangliosidosis fibroblasts

(2010) Katrin Fantur et al.   MOLECULAR GENETICS AND METABOLISM

(+)-proto-Quercitol, a natural versatile chiral building block for the synthesis of the α-glucosidase inhibitors, 5-amino-1,2,3,4-cyclohexanetetrols

(2009) Sumrit Wacharasindhu et al.   TETRAHEDRON LETTERS

A counterintuitive approach to treat enzyme deficiencies: use of enzyme inhibitors for restoring mutant enzyme activity

(2007) Jian-Qiang Fan   BIOLOGICAL CHEMISTRY