DLHex-DGJ, a novel derivative of 1-deoxygalactonojirimycin with pharmacological chaperone activity in human G(M1)-gangliosidosis fibroblasts.

DLHex-DGJ, a novel derivative of 1-deoxygalactonojirimycin with pharmacological chaperone activity in human G(M1)-gangliosidosis fibroblasts.

Item
http://www.wikidata.org/entity/Q39712268
The Molecular Basis of Pharmacological Chaperoning in Human α-GalactosidaseEvaluation 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.Candidate molecules for chemical chaperone therapy of GM1-gangliosidosis.Identification and characterization of pharmacological chaperones to correct enzyme deficiencies in lysosomal storage disorders.β-Glucosidase 2 (GBA2) activity and imino sugar pharmacology.Pharmacological chaperones for enzyme enhancement therapy in genetic diseases.Glycomimetic-based pharmacological chaperones for lysosomal storage disorders: lessons from Gaucher, GM1-gangliosidosis and Fabry diseases.N-Alkyl-, 1-C-Alkyl-, and 5-C-Alkyl-1,5-dideoxy-1,5-imino-(L)-ribitols as Galactosidase Inhibitors.1-Deoxy-D-galactonojirimycins with dansyl capped N-substituents as β-galactosidase inhibitors and potential probes for GM1 gangliosidosis affected cell lines.Fluorous iminoalditols act as effective pharmacological chaperones against gene products from GLB₁ alleles causing GM1-gangliosidosis and Morquio B disease.Chemical chaperone therapy: chaperone effect on mutant enzyme and cellular pathophysiology in β-galactosidase deficiency.Tuning protein folding in lysosomal storage diseases: the chemistry behind pharmacological chaperones.
P2860

DLHex-DGJ, a novel derivative of 1-deoxygalactonojirimycin with pharmacological chaperone activity in human G(M1)-gangliosidosis fibroblasts.

Item
http://www.wikidata.org/entity/Q39712268
description
2010 nî lūn-bûn
@nan
2010年の論文
@ja
2010年論文
@yue
2010年論文
@zh-hant
2010年論文
@zh-hk
2010年論文
@zh-mo
2010年論文
@zh-tw
2010年论文
@wuu
2010年论文
@zh
2010年论文
@zh-cn
name
DLHex-DGJ, a novel derivative ...... 1)-gangliosidosis fibroblasts.
@en
DLHex-DGJ, a novel derivative ...... 1)-gangliosidosis fibroblasts.
@nl
type
label
DLHex-DGJ, a novel derivative ...... 1)-gangliosidosis fibroblasts.
@en
DLHex-DGJ, a novel derivative ...... 1)-gangliosidosis fibroblasts.
@nl
prefLabel
DLHex-DGJ, a novel derivative ...... 1)-gangliosidosis fibroblasts.
@en
DLHex-DGJ, a novel derivative ...... 1)-gangliosidosis fibroblasts.
@nl
P1433
P1476
P2093
P304
P31
P356
P433
P478
P577
P698
P921
P356
P1433
P1476
DLHex-DGJ, a novel derivative ...... 1)-gangliosidosis fibroblasts.
@en
P2093
P304
P31
P356
10.1016/J.YMGME.2010.03.019
P433
P478
P577
P698
P921