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Native α-1,2-Fucosidase solution
Cat No.
NATE-0259
Description
Tissue alpha-L-fucosidase is an enzyme that in humans is encoded by the FUCA1 gene. 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, Native
Alias
FUCA1; FUCA
Package
vial of 0.004 unit
Form
buffered aqueous solution, Solution in 20 mM Tris-HCl, pH 7.5, 25 mM NaCl
Enzyme Commission Number
EC 3.2.1.51
Activity
> 0.4 units/mg protein
CAS No.
9037-65-4
Unit Definition
One unit will release 1.0 μmole of fucose from 2′-fucosyllactose per min at pH 5.0 at 37°C
Storage
2-8°C
Pathway
Lysosome, organism-specific biosystem; Other glycan degradation, organism-specific biosystem
Function
alpha-L-fucosidase activity; carbohydrate binding
Synonyms
α-1,2-Fucosidase; alpha-L-fucosidase; Alpha-Fucosidase; FUCA1; FUCA; 9037-65-4
Download
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