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BETA-N-ACETYLHEXOSAMINIDASE
Known as:
beta N Acetyl hexosaminidase
, N-Acetyl-beta-D-hexosaminidase
, beta N Acetylhexosaminidases
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A hexosaminidase specific for non-reducing N-acetyl-D-hexosamine residues in N-acetyl-beta-D-hexosaminides. It acts on GLUCOSIDES; GALACTOSIDES; and…
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National Institutes of Health
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Related topics
Related topics
16 relations
Gangliosidoses, GM2
Hexosaminidase A fr Ser
In Blood
Liver
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Broader (1)
Hexosaminidases
Narrower (1)
peptide O-linked N-acetylglucosamine-beta-N-acetylglucosaminidase
Papers overview
Semantic Scholar uses AI to extract papers important to this topic.
Highly Cited
1997
Highly Cited
1997
Apoptotic cell death in mouse models of GM2 gangliosidosis and observations on human Tay-Sachs and Sandhoff diseases.
J. Q. Huang
,
J. Trasler
,
Suleiman A. Igdoura
,
J. Michaud
,
N. Hanal
,
R. Gravel
Human Molecular Genetics
1997
Corpus ID: 9010634
Tay-Sachs and Sandhoff diseases are autosomal recessive neurodegenerative diseases resulting from the inability to catabolize GM2…
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Highly Cited
1983
Highly Cited
1983
The role of intermediate vesicles in the adsorptive endocytosis and transport of ligand to lysosomes by human fibroblasts
M. Merion
,
W. Sly
Journal of Cell Biology
1983
Corpus ID: 2870042
Recent work from several laboratories has suggested the participation of intermediate structures in the delivery of adsorbed…
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Highly Cited
1983
Highly Cited
1983
Phagocytosis of mast cell granules by cultured fibroblasts.
Fred M. Atkins
,
P. V. S. Rao
,
Marc M. Friedman
,
Dean D. Metcalfe
Journal of Immunology
1983
Corpus ID: 38333683
Highly Cited
1982
Highly Cited
1982
Complexing of glycolipids and their transfer between membranes by the activator protein for degradation of lysosomal ganglioside GM2.
E. Conzelmann
,
J. Burg
,
G. Stephan
,
K. Sandhoff
European Journal of Biochemistry
1982
Corpus ID: 2661318
The lysosomal degradation of ganglioside GM2 by hexosaminidase A depends on the presence of the specific activator protein which…
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Highly Cited
1981
Highly Cited
1981
The clinical spectrum of hexosaminidase deficiency diseases
W. Johnson
Neurology
1981
Corpus ID: 28720719
Hexosaminidase deficiency diseases or Gm2-gangliosidoses were originally described as infantile encephalopathies. Recently…
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Highly Cited
1979
Highly Cited
1979
Purification and characterization of an activator protein for the degradation of glycolipids GM2 and GA2 by hexosaminidase A.
E. Conzelmann
,
K. Sandhoff
Hoppe-Seyler´s Zeitschrift für physiologische…
1979
Corpus ID: 234714
The activator protein for the degradation of glycolipids GM2 and GA2 by hexosaminidase A was purified some 2 500-fold from normal…
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Highly Cited
1976
Highly Cited
1976
An activator stimulating the enzymic hydrolysis of sphingoglycolipids.
S. Li
,
Y. Li
Journal of Biological Chemistry
1976
Corpus ID: 6683082
An activator stimulating the enzymic hydrolysis of sphingoglycolipids has been purified from human liver. The purity of the…
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Highly Cited
1975
Highly Cited
1975
Defective lysosomal enzyme secretion in kidneys of Chediak-Higashi (beige) mice
E. J. Brandt
,
R. Elliott
,
R. Swank
Journal of Cell Biology
1975
Corpus ID: 1912480
The beige mouse is an animal model for the human Chediak-Higashi syndrome, a disease characterized by giant lysosomes in most…
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Highly Cited
1972
Highly Cited
1972
The demonstration of multiple heat stable forms of N-acetyl- -glucosaminidase in normal human serum.
Robert G. Price
,
N. Dance
Biochimica et Biophysica Acta
1972
Corpus ID: 42943305
Highly Cited
1968
Highly Cited
1968
Deficient hexosaminidase activity in an exceptional case of Tay-Sachs disease with additional storage of kidney globoside in visceral organs.
Konrad Sandhoff
,
U. Andreae
,
H. Jatzkewitz
Pathologia Europaea
1968
Corpus ID: 38329264
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