BETA N ACETYLHEXOSAMINIDASE A Meaning and
Definition
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Beta N-acetylhexosaminidase A is an enzyme that plays a crucial role in the hydrolysis of N-acetylhexosamine residues from complex carbohydrates and glycoproteins. It is a glycosyl hydrolase belonging to the family 20 of the glycoside hydrolase superfamily. This enzyme is primarily localized in lysosomes, which are membrane-bound cell organelles responsible for the breakdown of macromolecules.
The beta N-acetylhexosaminidase A enzyme is specifically designed to cleave the beta-glycosidic bond found between N-acetylhexosamine residues and other sugar molecules in glycoproteins or glycolipids. This hydrolysis process results in the release of individual sugars, which can be further metabolized by cells for energy production or other cellular functions.
Deficiency or dysfunction of beta N-acetylhexosaminidase A can lead to the accumulation of N-acetylhexosamines within lysosomes, causing the onset of a rare genetic disorder known as GM2 gangliosidosis or Tay-Sachs disease. In affected individuals, the enzyme's reduced activity or absence impairs the breakdown of glycolipids, leading to their accumulation in nerve cells. This accumulation ultimately results in severe neurological symptoms such as developmental regression, muscle weakness, loss of motor skills, and visual and hearing impairment.
In summary, beta N-acetylhexosaminidase A is an essential enzyme responsible for the degradation of N-acetylhexosamine residues from complex carbohydrates and glycoproteins. Its deficiency leads to the accumulation of these residues, giving rise to severe neurological symptoms in individuals affected by GM2 gangliosidosis.
Common Misspellings for BETA N ACETYLHEXOSAMINIDASE A
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