hexosaminidase A

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hexosaminidase A

[hek‚säs·ə′min·ə‚dās ′ā]
(biochemistry)
An enzyme which catalyzes the hydrolysis of the N-acetylgalactosamine residue from certain gangliosides.
References in periodicals archive ?
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.
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.
Juvenile GM2 gangliosidosis (AMB variant): inability to activate hexosaminidase A by activator protein.
1985) Motor neuron disease and adult hexosaminidase A deficiency in two families: evidence for multisystem degeneration.
Gaucher disease Glucocerebrosidase Neimann-Pick Sphingomyelinase Tay-Sachs [Beta] hexosaminidase Sandhoff [Beta] hexosaminidase Fabry [Alpha]-galactosidase Wolman Acid esterase Generalized gangliosidosis [Beta]-galactosidase
Salivary hexosaminidase in smoking alcoholics with bad periodontal and dental states.
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.
A glycine250 to aspartate substitution in the [alpha]-subunit of hexosaminidase A causes juvenile-onset Tay-Sachs disease in a Lebanese-Canadian Family.