Cat No.
NATE-1258
Description
Alpha-Fucosidase is an enzyme that breaks down fucose. Fucosidosis is an autosomal recessive lysosomal storage disease caused by defective alpha-L-fucosidase with accumulation of fucose in the tissues. Different phenotypes include clinical features such as neurologic deterioration, growth retardation, visceromegaly, and seizures in a severe early form; coarse facial features, angiokeratoma corporis diffusum, spasticity and delayed psychomotor development in a longer surviving form; and an unusual spondylometaphyseoepiphyseal dysplasia in yet another form.
Abbr
α(1-2) Fucosidase, Recombinant (Xanthomonas manihotis)
Alias
FUCA
Source
E. coli
Species
Xanthomonas manihotis
Concentration
20,000 units/ml
Molecular Mass
70000 daltons
Unit Definition
One unit is defined as the amount of enzyme required to cleave > 95% of the α-L-fucose from 1 nmol of Fucα1-2Galβ1-4Glc-7-amino-4-methyl-coumarin (AMC), in 1 hour at 37°C in a total reaction volume of 10 μl.
Synonyms
α-L-fucoside fucohydrolase; Alpha-Fucosidase; FUCA1; FUCA; EC 3.2.1.51
