Cat No.
NATE-1264
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
FUCA, Recombinant (Sweet almond tree)
Alias
FUCA
Source
Pichia pastoris
Species
Sweet almond tree
Form
20 mM Tris-HCl (pH 7.5), 50 mM NaCl and 1 mM EDTA
Bio-activity
75,000 units/mg
Concentration
4,000 units/ml
Molecular Mass
56000 daltons
Unit Definition
One unit is defined as the amount of enzyme required to cleave > 95% of the α-fucose from 1 nmol of Galβ1-4GlcNAcβ1-3(Fucα1-3)Galβ1- 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
