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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… 
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… 
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… 
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… 
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… 
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… 
Highly Cited
2005
Highly Cited
2005
Mutations in proteins that induce misfolding and proteasomal degradation are common causes of inherited diseases. Fabry disease… 
Highly Cited
2005
Highly Cited
2005
L-Enantiomers of 1-deoxynojirimycin (DNJ), 1-deoxymannojirimycin (manno-DNJ), 1-deoxyallonojirimycin (allo-DNJ), 1… 
Review
2003
Review
2003
  • N. Asano
  • Glycobiology
  • 2003
  • Corpus ID: 23044815
About 40 years have passed since the classical glycosidase inhibitor nojirimycin was discovered from the cultured broth of the… 
Highly Cited
2000
Highly Cited
2000
Fabry disease is a lysosomal storage disorder caused by deficient lysosomal alpha-galactosidase A (alpha-Gal A) activity…