FUCA1 Antibody

Alpha-L-fucosidase (EC 3.2.1.51) is a lysosomal enzyme involved in the degradation of fucose-containing glycoproteins and glycolipids. At least 2 separate polymorphic alpha-L-fucosidases are recognized in man: that in tissues, FUCA1, which is deficient in fucosidosis, and that in plasma, FUCA2. 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.

For short periods of storage (days) store at 4˚ C. For longer periods of storage, store FUCA1 antibody at -20˚ C. As with any antibody avoid repeat freeze-thaw cycles.

FUCA1 antibody can be used for detection of FUCA1 by ELISA at 1:312500. FUCA1 antibody can be used for detection of FUCA1 by western blot at 1 μ g/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.