FUCA1 Antikörper (N-Term)

Produktnummer ABIN633859

Produktdetails anti-FUCA1 Antikörper

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

Bindungsspezifität: N-Term

Reaktivität: Human, Maus, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser FUCA1 Antikörper ist unkonjugiert

Applikation: Western Blotting (WB)

Spezifität: FUCA1 antibody was raised against the N terminal of FUCA1

Aufreinigung: Affinity purified

Immunogen: FUCA1 antibody was raised using the N terminal of FUCA1 corresponding to a region with amino acids PSPVSWNWNSKDVGPHRDLVGELGTALRKRNIRYGLYHSLLEWFHPLYLL

Anwendungsinformationen

Applikationshinweise: WB: 1 µg/mL
Optimal conditions should be determined by the investigator.

Kommentare:

FUCA1 Blocking Peptide, catalog no. 33R-7363, is also available for use as a blocking control in assays to test for specificity of this FUCA1 antibody

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Lyophilized

Rekonstitution: Lyophilized powder. Add distilled water for a 1 mg/mL concentration of FUCA1 antibody in PBS

Konzentration: Lot specific

Buffer: PBS

Handhabung: Avoid repeated freeze/thaw cycles.
Dilute only prior to immediate use.

Lagerung: 4 °C/-20 °C

Informationen zur Lagerung: Store at 2-8 °C for short periods. For longer periods of storage, store at -20 °C.

Details zu FUCA1

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

Andere Bezeichnung: FUCA1 (FUCA1 Produkte)

Hintergrund: 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 recognised 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.

Molekulargewicht: 54 kDa (MW of target protein)

Pathways: Glycosaminoglycan Metabolic Process