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1-deoxy-galactonojirimycin

Known as: 1-deoxygalacto-nojirimycin, 1-deoxygalactonojirimycin 
 

Topic mentions per year

Topic mentions per year

2000-2016
024620002016

Related topics

Related topics

1 relation
migalastat

Related mentions per year

Related mentions per year

2000-2017
20002005201020152020
1-deoxy-galactonojirimycin
migalastat

Papers overview

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2012
2012
Co-administration With the Pharmacological Chaperone AT1001 Increases Recombinant Human α-Galactosidase A Tissue Uptake and Improves Substrate Reduction in Fabry Mice
Fabry disease is an X-linked lysosomal storage disorder (LSD) caused by mutations in the gene (GLA) that encodes the lysosomal… (More)
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2011
2011
A Pharmacogenetic Approach to Identify Mutant Forms of α-Galactosidase A that Respond to a Pharmacological Chaperone for Fabry Disease
Fabry disease is caused by mutations in the gene (GLA) that encodes α-galactosidase A (α-Gal A). The iminosugar AT1001 (GR181413A… (More)
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2010
2010
The pharmacological chaperone 1-deoxygalactonojirimycin reduces tissue globotriaosylceramide levels in a mouse model of Fabry disease.
Fabry disease is an X-linked lysosomal storage disorder caused by a deficiency in alpha-galactosidase A (alpha-Gal A) activity… (More)
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2009
2009
The pharmacological chaperone 1-deoxygalactonojirimycin increases α-galactosidase A levels in Fabry patient cell lines
Fabry disease is an X-linked lysosomal storage disorder caused by mutations in the gene encoding α-galactosidase A (α-Gal A… (More)
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2008
2008
Novel mutations of the GLA gene in Japanese patients with Fabry disease and their functional characterization by active site specific chaperone.
Fabry disease is an X-linked recessive inborn metabolic disorder caused by a deficiency of the lysosomal enzyme alpha… (More)
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2007
2007
Mutant alpha-galactosidase A enzymes identified in Fabry disease patients with residual enzyme activity: biochemical characterization and restoration of normal intracellular processing by 1…
Fabry disease is a lysosomal storage disorder caused by the deficiency of alpha-Gal A (alpha-galactosidase A) activity. In order… (More)
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2007
2007
Inhibition of raffinose oligosaccharide breakdown delays germination of pea seeds.
Raffinose family oligosaccharides (RFOs) are almost ubiquitous in seeds and have been hypothesized to constitute an important… (More)
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2006
2006
Pharmacological chaperone corrects lysosomal storage in Fabry disease caused by trafficking-incompetent variants.
Fabry disease is a lysosomal storage disorder caused by deficiency of alpha-galactosidase A (alpha-Gal A) resulting in lysosomal… (More)
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2005
2005
A synthetic chaperone corrects the trafficking defect and disease phenotype in a protein misfolding disorder.
Mutations in proteins that induce misfolding and proteasomal degradation are common causes of inherited diseases. Fabry disease… (More)
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2000
2000
In vitro inhibition and intracellular enhancement of lysosomal alpha-galactosidase A activity in Fabry lymphoblasts by 1-deoxygalactonojirimycin and its derivatives.
Fabry disease is a lysosomal storage disorder caused by deficient lysosomal alpha-galactosidase A (alpha-Gal A) activity… (More)
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