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Hexosaminidase (EC 3.2.1.52, β-acetylaminodeoxyhexosidase, N-acetyl-β-D-hexosaminidase, N-acetyl-β-hexosaminidase, N-acetyl hexosaminidase, β-hexosaminidase, β-acetylhexosaminidinase, β-D-N-acetylhexosaminidase, β-N-acetyl-D-hexosaminidase, β-N-acetylglucosaminidase, hexosaminidase A, N-acetylhexosaminidase, β-D-hexosaminidase) is an enzyme involved in the hydrolysis of terminal N ...
Hexosaminidase A (alpha polypeptide), also known as HEXA, is an enzyme that in humans is encoded by the HEXA gene, located on the 15th chromosome. [ 5 ] [ 6 ] Hexosaminidase A and the cofactor G M2 activator protein catalyze the degradation of the G M2 gangliosides and other molecules containing terminal N-acetyl hexosamines. [ 7 ]
There are 3 forms of beta-hexosaminidase: hexosaminidase A is a trimer, with one alpha, one beta-A and one beta-B chain; hexosaminidase B is a tetramer of two beta-A and two beta-B chains; and hexosaminidase S is a homodimer of alpha chains. The two beta chains are derived from the cleavage of a precursor.
Beta-hexosaminidase subunit beta is an enzyme that in humans is encoded by the HEXB gene. [ 5 ] [ 6 ] [ 7 ] Hexosaminidase B is the beta subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl ...
Tay–Sachs disease is caused by insufficient activity of the enzyme hexosaminidase A. Hexosaminidase A is a vital hydrolytic enzyme, found in the lysosomes, that breaks down sphingolipids. When hexosaminidase A is no longer functioning properly, the lipids accumulate in the brain and interfere with normal biological processes.
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Sandhoff disease is a lysosomal genetic, lipid storage disorder caused by the inherited deficiency to create functional beta-hexosaminidases A and B. [1] [2] These catabolic enzymes are needed to degrade the neuronal membrane components, ganglioside GM2, its derivative GA2, the glycolipid globoside in visceral tissues, [1] and some oligosaccharides.
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