84-3498-63 [取扱停止]GLA, NT (Alpha-galactosidase A, Alpha-D-galactoside Galactohydrolase, Alpha-D-galactosidase A, Agalsidase, Melibiase) (FITC) 200ul G2035-48-FITC
84-3498-63 [取扱停止]GLA, NT (Alpha-galactosidase A, Alpha-D-galactoside Galactohydrolase, Alpha-D-galactosidase A, Agalsidase, Melibiase) (FITC) 200ul G2035-48-FITC
GLA is a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins.
This enzyme predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose.
A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties.
Applications:Suitable for use in FLISA, Flow Cytometry (Not Tested), Immunocytochemistry/Immunofluorescence, Immunohistochemistry and Western Blot.
Other applications not tested.
Recommended Dilution:Immunohistochemistry: Formalin-fixed, paraffin-emnbedded sectionsOptimal dilutions to be determined by the researcher.
Storage and Stability:Store product at 4°C if to be used immediately within two weeks.
For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20°C.
Aliquots are stable at -20°C for 12 months after receipt.
Dilute required amount only prior to immediate use.
Further dilutions can be made in assay buffer.
Caution: FITC conjugates are sensitive to light.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Note: Applications are based on unconjugated antibody.
仕様
Size:200ul
Host:rabbit
Source Antibody:human
Grade:Affinity Purified
Purity:Purified by Protein A/G affinity chromatography.
Form:Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein isothiocyanate (FITC).
Specificity:Recognizes human GLA.
Isotype:IgG
Calc Applications Abbrev:FLISA IC IF IHC WB
Calc Crossreactivity:Hu
Immunogen:KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 83-112 in the N-terminal region of human GLA, UniPot Accession #P06280.