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.1) Venditti, J.J., (2007) Mol. Reprod. Dev. 74 (6), 758-766.
Immunogen
Antibody produced in rabbits immunized with a synthetic peptide corresponding a region of human FUCA1.
Key Feature
Clonality
Polyclonal
Host Species
Rabbit
Tested Applications
ELISAWB
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 μg/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.:
Antibody used in WB on Human Liver at 0.2-1 ug/ml.
Positive Control
1) Cat. No. XBL-10409 - Fetal Liver Tissue Lysate
Application Notes
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 μg/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.:
Additional Information
Form
Liquid
Storage Instructions
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.
Storage Buffer
Antibody is lyophilized in PBS buffer with 2% sucrose. Add 50 μL of distilled water. Final antibody concentration is 1 mg/mL.