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FUCA1 Antibody (14-J2) is a mouse monoclonal IgG2b antibody that detects FUCA1 in human samples through applications such as western blotting (WB), immunoprecipitation (IP), and enzyme-linked immunosorbent assay (ELISA). FUCA1 is a 466 amino acid membrane- and seminal-associated isozyme belonging to the glycosyl hydrolase 29 family, functioning as a homotetramer responsible for hydrolyzing and reducing the carbohydrate moieties of glycoproteins across various tissues. The importance of FUCA1 lies in its role in lysosomal metabolism; defects in the gene encoding FUCA1 lead to fucosidosis, an autosomal recessive disorder characterized by the accumulation of fucose-containing glycolipids and glycoproteins. This lysosomal storage disease manifests with severe symptoms, including neurologic deterioration, growth retardation, visceromegaly, and seizures. Notably, early onset fucosidosis can result in distinctive clinical features such as coarse facial features, angiokeratoma corporis diffusum, spasticity, delayed psychomotor development, and a unique form of spondylometaphyseoepiphyseal dysplasia. FUCA1 (14-J2) antibody is essential for researchers studying the implications of FUCA1 in metabolic disorders and can aid in the exploration of therapeutic strategies targeting fucosidosis.
Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
FUCA1 Antibody (14-J2) | sc-100530 | 100 µg/ml | $339.00 |