FUCA1 anticorps

N° du produit ABIN2459727

Détail du produit anti-FUCA1 anticorps

Antigène: FUCA1 (Fucosidase, alpha-L- 1, Tissue (FUCA1))

Reactivité: Humain, Souris, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp FUCA1 est non-conjugé

Application: Western Blotting (WB), ELISA

Purification: Antibody is purified by peptide affinity chromatography method.

Immunogène: Antibody produced in rabbits immunized with a synthetic peptide corresponding a region of human FUCA1.

Information d'application

Indications d'application: 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.

Restrictions: For Research Use only

Stockage

Format: Lyophilized

Reconstitution: Add 50 ?L of distilled water. Final antibody concentration is 1 mg/mL.

Concentration: 1 mg/mL

Buffer: Antibody is lyophilized in PBS buffer with 2 % sucrose.

Conseil sur la manipulation: As with any antibody avoid repeat freeze-thaw cycles.

Stock: 4 °C/-20 °C

Stockage commentaire: For short periods of storage (days) store at 4 °C. For longer periods of storage, store FUCA1 antibody at -20 °C.

Détails sur FUCA1

Antigène: FUCA1 (Fucosidase, alpha-L- 1, Tissue (FUCA1))

Autre désignation: FUCA1 (FUCA1 Produits)

Synonymes: anticorps FUCA, anticorps FUCA1, anticorps 0610006A03Rik, anticorps 9530055J05Rik, anticorps Afuc, anticorps Fuca, anticorps fuca, anticorps fuca1, anticorps ATFUC1, anticorps F24D13.11, anticorps F24D13_11, anticorps alpha-L-fucosidase 1, anticorps wu:fb02a09, anticorps zgc:101116, anticorps alpha-L-fucosidase 1, anticorps fucosidase, alpha-L- 1, tissue, anticorps alpha-L-fucosidase 1 L homeolog, anticorps alpha-L-fucosidase 1, tandem duplicate 2, anticorps FUCA1, anticorps Fuca1, anticorps fuca1.L, anticorps fuca1, anticorps FUC1, anticorps LOC9317020, anticorps LOC100285202, anticorps fuca1.2

Sujet: 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.

Poids moléculaire: 54 kDa

ID gène: 2517

NCBI Accession: NP_000138

UniProt: P04066

Pathways: Glycosaminoglycan Metabolic Process