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.

hex·os·a·min·i·dase

(heks-ōs'ă-min'i-dās)
General term for enzymes cleaving N-acetylhexose residues from gangliosidelike oligosaccharides.
References in periodicals archive ?
Withers, "Aspartate 313 in the Streptomyces plicatus hexosaminidase plays a critical role in substrate-assisted catalysis by orienting the 2-acetamido group and stabilizing the transition state," Journal of Biological Chemistry, vol.
(1,5) In Sandhoff's disease, total hexosaminidase activity is reduced to less than 2 - 3% of normal, unlike Tay Sachs, where total hexosaminidase activity is preserved due to functional [beta]-hexosaminidase B activity.
Conditions associated with hepatosplenomegaly like Gaucher disease, hexosaminidase A deficiency, Sandhoff disease, Niemann-Pick disease type C, Wolman disease, the mucopolysaccharidoses.
(1985) Motor neuron disease and adult hexosaminidase A deficiency in two families: evidence for multisystem degeneration.
The most known Jewish genetic disease is Tay-Sachs, caused by a deficiency of the enzyme hexosaminidase A.
Gaucher disease Glucocerebrosidase Neimann-Pick Sphingomyelinase Tay-Sachs [Beta] hexosaminidase Sandhoff [Beta] hexosaminidase Fabry [Alpha]-galactosidase Wolman Acid esterase Generalized gangliosidosis [Beta]-galactosidase
Schwartz, "Hexosaminidase: a biochemical marker for necrotizing enterocolitis in the preterm infant," The American Journal of Surgery, vol.
Dashed line indicates the level of [beta]- hexosaminidase release induced by stimulation with 10ng/mL DNP-HSA alone (c).
Hexosaminidase fluorometric assays for diagnosis of Tay-Sachs disease have been around for many years and make use of a pair of assays with heat treatment to tease out HexA and HexB activities.