hexosaminidase

hexosaminidase
General term for enzymes cleaving N-acetylhexose ( e.g., N-acetylglucosamine) residues from gangliosidelike oligosaccharides. At least four specific enzymes carrying out this type of reaction are known: α-N-acetyl-d-galactosaminidase, α-N-acetyl-d-glucosaminidase, β-N-acetyl-d-h., and β-N-acetyl-d-galactosaminidase, each being specific for the configuration and type of sugar included in the name.
- h. A a hydrolytic enzyme that acts on ganglioside GM2, producing N-acetyl-d-galactosamine and ganglioside GM3; a deficiency of this enzyme is associated with Tay-Sachs disease.
- h. B a hydrolytic enzyme that acts on ganglioside GM1, producing ganglioside GM1 and galactose, as well as on globoside, producing N-acetylgalactosamine and trihexosylceramide; a deficiency of this enzyme is associated with Sandhoff disease.

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hex·os·a·min·i·dase .hek-.säs-ə-'min-ə-.dās, -.dāz n either of two hydrolytic enzymes that catalyze the splitting off of a hexose from a ganglioside and are deficient in some metabolic diseases:
a) HEXOSAMINIDASE A
b) HEXOSAMINIDASE B

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hex·os·amin·i·dase (hek″sōs-ə-minґĭ-dās) 1. any of the enzymes that cleave hexosamine or N-acetylhexosamine residues from gangliosides or other glycosides. Specific enzymes are named for the specific amino sugar and linkage that are potential substrates (e.g., α-N-acetylglucosaminidase). 2. β-N-acetylhexosaminidase.

Medical dictionary. 2011.

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  • hexosaminidase B — n the more thermostable hexosaminidase that is deficient in Sandhoff s disease but present in elevated quantities in Tay Sachs disease called also hex B * * * β N acetylhexosaminidase, isozyme B …   Medical dictionary

  • hexosaminidase — hexosaminidase. См. гексозаминидаза. (Источник: «Англо русский толковый словарь генетических терминов». Арефьев В.А., Лисовенко Л.А., Москва: Изд во ВНИРО, 1995 г.) …   Молекулярная биология и генетика. Толковый словарь.

  • Hexosaminidase — β N acetylhexosaminidase Hexosaminidase A (Hex A) Identifiers EC number 3.2.1.52 …   Wikipedia

  • Hexosaminidase A — Deficiency of this enzyme causes Tay Sachs disease, a progressive, fatal neurologic disorder concentrated in people of European Jewish (Ashkenazi) descent. * * * hexosaminidase A n the more thermolabile hexosaminidase that is deficient in both… …   Medical dictionary

  • hexosaminidase — noun Etymology: hexose + amino + ide + ase Date: 1969 either of two hydrolytic enzymes that catalyze the splitting off of a hexose from a ganglioside and are deficient in some metabolic diseases (as Tay Sachs disease) …   New Collegiate Dictionary

  • hexosaminidase — Enzyme involved in the metabolism of gangliosides. Deficient in Tay Sachs disease …   Dictionary of molecular biology

  • hexosaminidase — noun Any enzyme that catalyzes the hydrolysis of terminal hexosamine residues in hexosaminides …   Wiktionary

  • hexosaminidase — hex·os·a·min·i·dase …   English syllables

  • hexosaminidase — ˌhekˌsäsəˈminə̇ˌdās, āz noun ( s) Etymology: hexosamine + ide (I) + ase : either of two hydrolytic enzymes that catalyze the splitting off of a hexose from a ganglioside and are deficient in some metabolic diseases (as Tay Sachs disease) …   Useful english dictionary

  • Hexosaminidase A deficiency — Lack of an enzyme that causes Tay Sachs disease. Hex A deficiency results in failure to process a lipid (a fat) which accumulates and is deposited in the brain and other tissues, to their detriment. The lipid that is deposited is called GM2… …   Medical dictionary

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