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1-deoxy-galactonojirimycin

Known as: 1-deoxygalacto-nojirimycin, 1-deoxygalactonojirimycin 
National Institutes of Health

Related topics

Related topics

1 relation
migalastat

Papers overview

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2016
2016
Substrate and Substrate-Mimetic Chaperone Binding Sites in Human α-Galactosidase A Revealed by Affinity-Mass Spectrometry
AbstractFabry disease (FD) is a rare metabolic disorder of a group of lysosomal storage diseases, caused by deficiency or reduced… 
Review
2013
Review
2013
[Structural basis for β-galactosidase associated with lysosomal disease].
  • Toshiyuki Shimizu
  • Yakugaku zasshi : Journal of the Pharmaceutical…
  • 2013
  • Corpus ID: 8381880
G(M1)-gangliosidosis and Morquio B are rare lysosomal storage diseases associated with a neurodegenerative disorder or dwarfism… 
2013
2013
A bicyclic 1-deoxygalactonojirimycin derivative as a novel pharmacological chaperone for GM₁ gangliosidosis
2012
2012
Using bicyclic derivatives of 1-deoxygalactonojirimycin in the preparation of a medicament for the treatment of diseases related to lysosomal enzymes beta-galactosidase mutant human
The present invention relates to a use of a compound of formula (I) # **** **** IMAGE # or a salt thereof, wherein: #R represents… 
2011
2011
Molecular mechanism for stabilization of a mutant α-galactosidase A involving M51I amino acid substitution by imino sugars.
Highly Cited
2010
Highly Cited
2010
DLHex-DGJ, a novel derivative of 1-deoxygalactonojirimycin with pharmacological chaperone activity in human G(M1)-gangliosidosis fibroblasts.
2010
2010
Synthesis of lipophilic 1-deoxygalactonojirimycin derivatives as D-galactosidase inhibitors
N-Alkylation at the ring nitrogen of the D-galactosidase inhibitor 1-deoxygalactonojirimycin with a functionalised C6 alkyl chain… 
2006
2006
Beta-galactosidase deficiency: an approach to chaperone therapy.
We propose a new molecular therapeutic approach to lysosomal diseases with severe neurological manifestations. Some low-molecular… 
Highly Cited
2003
Highly Cited
2003
Concise and highly stereocontrolled synthesis of 1-deoxygalactonojirimycin and its congeners using dioxanylpiperidene, a promising chiral building block.
[reaction: see text] A concise and stereoselective synthesis of the chiral building block, dioxanylpiperidene 4 as a precursor… 
2001
2001
alpha- and beta-homogalactonojirimycins (alpha- and beta-homogalactostatins): synthesis and further biological evaluation.
The homoiminosugars alpha- and beta-homogalactonojirimycins were prepared from a common intermediate, tetra-O-benzyl-D-galacto… 
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