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1-deoxy-galactonojirimycin
Known as:
1-deoxygalacto-nojirimycin
, 1-deoxygalactonojirimycin
National Institutes of Health
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Related topics
1 relation
migalastat
Papers overview
Semantic Scholar uses AI to extract papers important to this topic.
Highly Cited
2012
Highly Cited
2012
Co-administration With the Pharmacological Chaperone AT1001 Increases Recombinant Human α-Galactosidase A Tissue Uptake and Improves Substrate Reduction in Fabry Mice
E. Benjamin
,
R. Khanna
,
+13 authors
K. Valenzano
Molecular therapy : the journal of the American…
2012
Corpus ID: 8035455
Fabry disease is an X-linked lysosomal storage disorder (LSD) caused by mutations in the gene (GLA) that encodes the lysosomal…
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Highly Cited
2011
Highly Cited
2011
Crystal Structure of Human β-Galactosidase
U. Ohto
,
K. Usui
,
T. Ochi
,
K. Yuki
,
Y. Satow
,
Toshiyuki Shimizu
The Journal of Biological Chemistry
2011
Corpus ID: 1736030
Background: Deficiencies in β-d-galactosidase cause lysosomal storage diseases. Results: This is the first report to describe the…
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Highly Cited
2010
Highly Cited
2010
The pharmacological chaperone 1-deoxygalactonojirimycin reduces tissue globotriaosylceramide levels in a mouse model of Fabry disease.
R. Khanna
,
R. Soska
,
+10 authors
K. Valenzano
Molecular therapy : the journal of the American…
2010
Corpus ID: 24704066
Fabry disease is an X-linked lysosomal storage disorder caused by a deficiency in alpha-galactosidase A (alpha-Gal A) activity…
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Highly Cited
2009
Highly Cited
2009
The pharmacological chaperone 1-deoxygalactonojirimycin increases α-galactosidase A levels in Fabry patient cell lines
E. Benjamin
,
J. Flanagan
,
+9 authors
K. Valenzano
Journal of Inherited Metabolic Disease
2009
Corpus ID: 12629461
SummaryFabry disease is an X-linked lysosomal storage disorder caused by mutations in the gene encoding α-galactosidase A (α-Gal…
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Highly Cited
2007
Highly Cited
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…
S. Ishii
,
Hui-Hwa Chang
,
+4 authors
Jian‐Qiang Fan
The Biochemical journal
2007
Corpus ID: 41320983
Fabry disease is a lysosomal storage disorder caused by the deficiency of alpha-Gal A (alpha-galactosidase A) activity. In order…
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Highly Cited
2006
Highly Cited
2006
Pharmacological chaperone corrects lysosomal storage in Fabry disease caused by trafficking-incompetent variants.
G. Yam
,
N. Bosshard
,
C. Zuber
,
B. Steinmann
,
J. Roth
American journal of physiology. Cell physiology
2006
Corpus ID: 2393358
Fabry disease is a lysosomal storage disorder caused by deficiency of alpha-galactosidase A (alpha-Gal A) resulting in lysosomal…
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Highly Cited
2005
Highly Cited
2005
A synthetic chaperone corrects the trafficking defect and disease phenotype in a protein misfolding disorder
G. Yam
,
C. Zuber
,
J. Roth
FASEB journal : official publication of the…
2005
Corpus ID: 8460247
Mutations in proteins that induce misfolding and proteasomal degradation are common causes of inherited diseases. Fabry disease…
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Highly Cited
2005
Highly Cited
2005
Biological properties of D- and L-1-deoxyazasugars.
A. Kato
,
N. Kato
,
+8 authors
N. Asano
Journal of medicinal chemistry
2005
Corpus ID: 36649255
L-Enantiomers of 1-deoxynojirimycin (DNJ), 1-deoxymannojirimycin (manno-DNJ), 1-deoxyallonojirimycin (allo-DNJ), 1…
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Review
2003
Review
2003
Glycosidase inhibitors: update and perspectives on practical use.
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…
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Highly Cited
2000
Highly Cited
2000
In vitro inhibition and intracellular enhancement of lysosomal alpha-galactosidase A activity in Fabry lymphoblasts by 1-deoxygalactonojirimycin and its derivatives.
N. Asano
,
S. Ishii
,
+5 authors
J. Q. Fan
European journal of biochemistry
2000
Corpus ID: 20226885
Fabry disease is a lysosomal storage disorder caused by deficient lysosomal alpha-galactosidase A (alpha-Gal A) activity…
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