Background: Alpha-L-fucosidase (FUCA, FUCA1) is responsible for hydrolyzing the alpha-1,6-linked fucose joined to the reducing-end N-acetylglucosamine of the carbohydrate moieties of glycoproteins. FUCA is a 466 amino acid membrane- and seminal-associated isozyme that is a member of the glycosyl hydrolase 29 family. FUCA functions as a homotetramer and is responsible for hydrolyzing and reducing the carbohydrate moieties of glycoproteins in various tissues. Defects in the gene encoding FUCA result in fucosidosis, an autosomal recessive disorder caused by an accumulation of fucose-containing glycolipids and glycoproteins. Fucosidosis, a lysosomal storage disease, is characterized by neurologic deterioration, growth retardation, visceromegaly and seizures. Early onset of fucosidosis causes coarse facial features, angiokeratoma corporis diffusum, spasticity, delayed psychomotor development and an unusual spondylometaphyseoepiphyseal dysplasia.
Description: Rabbit polyclonal to FUCA
Immunogen: KLH conjugated synthetic peptide derived from FUCA
Specificity: ·Reacts with Human, Mouse, Pig, Cow, Dog and Rat.
·Isotype: IgG
Application: ·Western blotting: 1/100-500. Predicted Mol wt: 54 kDa;
·Immunohistochemistry (Paraffin/frozen tissue section): 1/50-200;
·Immunocytochemistry/Immunofluorescence: 1/100;
·Immunoprecipitation: 1/50;
·ELISA: 1/500;
·Optimal working dilutions must be determined by the end user.
