hexosaminidase


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hex·os·a·min·i·dase

(hek'sōs-ă-min'i-dās),
General term for enzymes hydrolyzing N-acetylhexose (for example, 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-hexosaminidase, and β-N-acetyl-d-galactosaminidase, each being specific for the configuration and type of sugar included in the name.

hexosaminidase

/hex·os·amin·i·dase/ (hek″sōs-ah-min´ĭ-dās)
1. any of the enzymes that cleave hexosamines or acetylated hexosamines from gangliosides or other glycosides.
2. a specific hexosaminidase acting on keratan sulfate and ganglioside GM2 and related compounds.

hex·os·a·min·i·dase

(heks-ōs'ă-min'i-dās)
General term for enzymes cleaving N-acetylhexose residues from gangliosidelike oligosaccharides.

hexosaminidase

an enzyme that catalyzes the cleavage of hexose from ganglioside GM2. Occurs in two forms, A and B. Deficiency occurs in GM2gangliosidosis of dogs, cats and pigs.
References in periodicals archive ?
The percentage of ([beta]- hexosaminidase release in each tube was calculated and taken as a measure of mast cell activation.
Juvenile GM2 gangliosidosis (AMB variant): inability to activate hexosaminidase A by activator protein.
NO PREVENTING THIS UNTREATABLE AND RARE DISEASE* Tay-Sachs is caused by a deficiency of a protein called hexosaminidase A.
Plasma hexosaminidase levels were significantly decreased.
Sandhoff disease springs from the lack of the enzyme hexosaminidase (hex), which dears excess lipids from the brain.
Tay-Sachs disease is characterized by absence of hexosaminidase A, an enzyme that breaks down GM2-ganglioside.
The major defect in Ashkenazi Jews with Tay Sachs Disease is an insertion in the gene for the alpha-chain of beta hexosaminidase.
This condition arises from an error of lipid metabolism in which a defect in hexosaminidase A results in accumulation of ganglioside [GM.
Gaucher disease Glucocerebrosidase Neimann-Pick Sphingomyelinase Tay-Sachs [Beta] hexosaminidase Sandhoff [Beta] hexosaminidase Fabry [Alpha]-galactosidase Wolman Acid esterase Generalized gangliosidosis [Beta]-galactosidase
The reaction product was measured at 405 nm ([beta]-hexosaminidase) or 450 Om (histamine) using a micropiate reader (Tecan, Austria), and percentage of [beta]- hexosaminidase or histamine release was calculated as follows: release supernatant/(release supernatant + release pellet) x 100.