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Gangliosidoses, GM2

Known as: GM2, Gangliosidoses, Gangliosidose, GM2, Gangliosidoses GM2 
A group of recessively inherited diseases characterized by the intralysosomal accumulation of G(M2) GANGLIOSIDE in the neuronal cells. Subtypes… Expand
National Institutes of Health

Papers overview

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Highly Cited
2004
Highly Cited
2004
Lysosomal storage diseases (LSDs) are a group of inherited metabolic disorders individually considered as rare, and few data on… Expand
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Highly Cited
2004
Highly Cited
2004
Mice containing a disruption of the Hexb gene have provided a useful model system for the study of the human lysosomal storage… Expand
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Highly Cited
2003
Highly Cited
2003
Mouse models of the GM2 gangliosidoses [Tay-Sachs, late onset Tay-Sachs (LOTS), Sandhoff] and GM1 gangliosidosis have been… Expand
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Review
1999
Review
1999
  • D. Mahuran
  • Biochimica et biophysica acta
  • 1999
  • Corpus ID: 25277998
The hydrolysis of GM2-ganglioside is unusual in its requirements for the correct synthesis, processing, and ultimate combination… Expand
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Highly Cited
1998
Highly Cited
1998
The GM2 gangliosidoses are a group of severe, neurodegenerative conditions that include Tay-Sachs disease, Sandhoff disease, and… Expand
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1992
1992
Abstract: By using a sensitive method, we assayed lysocom‐pounds of gangliosides and asialogangliosides in tissues from four… Expand
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Review
1991
Review
1991
The authors describe the clinical phenotypes of hexosaminidase deficiencies (GM2 gangliosidosis). The symptoms, differently… Expand
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1990
1990
In 13 patients, the GM2 gangliosidoses, Sandhoff disease and Tay-Sachs disease, were found to be constantly associated with… Expand
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Highly Cited
1990
Highly Cited
1990
The lysosomal catabolism of sulfatide requires arylsulfatase A and a specific sphingolipid activator protein, SAP-1. While most… Expand
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Highly Cited
1985
Highly Cited
1985
Ganglioside GM2, 3H-labeled in the sphingoid base, was added to the culture medium of normal and GM2 gangliosidosis fibroblasts… Expand
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