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1-deoxy-galactonojirimycin

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

Papers overview

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Highly Cited
2012
Highly Cited
2012
Fabry disease is an X-linked lysosomal storage disorder (LSD) caused by mutations in the gene (GLA) that encodes the lysosomal… Expand
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Highly Cited
2011
Highly Cited
2011
Background: Deficiencies in β-d-galactosidase cause lysosomal storage diseases. Results: This is the first report to describe the… Expand
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Highly Cited
2010
Highly Cited
2010
Fabry disease is an X-linked lysosomal storage disorder caused by a deficiency in alpha-galactosidase A (alpha-Gal A) activity… Expand
Highly Cited
2009
Highly Cited
2009
SummaryFabry disease is an X-linked lysosomal storage disorder caused by mutations in the gene encoding α-galactosidase A (α-Gal… Expand
Highly Cited
2007
Highly Cited
2007
Fabry disease is a lysosomal storage disorder caused by the deficiency of alpha-Gal A (alpha-galactosidase A) activity. In order… Expand
Highly Cited
2006
Highly Cited
2006
Fabry disease is a lysosomal storage disorder caused by deficiency of alpha-galactosidase A (alpha-Gal A) resulting in lysosomal… Expand
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Highly Cited
2005
Highly Cited
2005
L-Enantiomers of 1-deoxynojirimycin (DNJ), 1-deoxymannojirimycin (manno-DNJ), 1-deoxyallonojirimycin (allo-DNJ), 1… Expand
Highly Cited
2005
Highly Cited
2005
Mutations in proteins that induce misfolding and proteasomal degradation are common causes of inherited diseases. Fabry disease… Expand
Highly Cited
2003
Highly Cited
2003
[reaction: see text] A concise and stereoselective synthesis of the chiral building block, dioxanylpiperidene 4 as a precursor… Expand
Highly Cited
2000
Highly Cited
2000
Fabry disease is a lysosomal storage disorder caused by deficient lysosomal alpha-galactosidase A (alpha-Gal A) activity… Expand