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Hexosaminidase A

Known as: Hex A, Beta-N-acetylhexosaminidase.A, beta N Acetylhexosaminidase A 
A mammalian beta-hexosaminidase isoform that is a heteromeric protein comprized of both hexosaminidase alpha and hexosaminidase beta subunits… Expand
National Institutes of Health

Papers overview

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Highly Cited
2006
Highly Cited
2006
Lysosomal beta-hexosaminidase A (Hex A) is essential for the degradation of GM2 gangliosides in the central and peripheral… Expand
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Highly Cited
2003
Highly Cited
2003
In humans, two major beta-hexosaminidase isoenzymes exist: Hex A and Hex B. Hex A is a heterodimer of subunits alpha and beta (60… Expand
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Highly Cited
1994
Highly Cited
1994
The cDNA encoding GM2 activator was expressed in the Escherichia coli/pT7-7 system. The yield of the GM2 activator with greater… Expand
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1993
1993
Deficient activity of beta-hexosaminidase A (Hex A), resulting from mutations in the HEXA gene, typically causes Tay-Sachs… Expand
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Highly Cited
1992
Highly Cited
1992
Deficiency of beta-hexosaminidase A (Hex A) activity typically results in Tay-Sachs disease. However, healthy subjects found to… Expand
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Highly Cited
1991
Highly Cited
1991
Ganglioside GD1a-GalNAc was isolated from Tay-Sachs brain, tritium-labeled in its sphingosine moiety, and its enzymic degradation… Expand
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Highly Cited
1986
Highly Cited
1986
Deficiency of hexosaminidase A (Hex A) in adults was found in 15 individuals from nine unrelated Ashkenazi families; 14… Expand
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Highly Cited
1982
Highly Cited
1982
Summary: A patient is described whose clinical course and pathologic features, including massive brain storage of GM2 ganglioside… Expand
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Highly Cited
1978
Highly Cited
1978
Human kidney extracts heated to 60 degrees and devoid of hexosaminidase activity (2-acetamido-2-deoxy-beta-D-glucoside… Expand
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Highly Cited
1976
Highly Cited
1976
To determine whether ligand-receptor interactions could engender the selective uptake by deficient cells of enzyme-laden… Expand
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