DATASHEET
Host:
Rabbit
Target Protein:
Galactosidase alpha
Immunogen Range:
101-200/429
Clonality:
Polyclonal
Isotype:
IgG
Entrez Gene:
2717
Swiss Prot:
P06280
Source:
KLH conjugated synthetic peptide derived from human Galactosidase alpha
Purification:
Purified by Protein A.
Storage Buffer:
Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Storage:
Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
Background:
Galactosidase alpha is involved in the hydrolysis of terminal, non reducing alpha D galactose residues in alpha D galactosides, including galactose oligosaccharides, galactomannans and galactohydrolase. Defects in GLA are the cause of Fabry's disease (FD). FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities.
Conjugation:
ALEXA FLUOR® 488
Excitation/ Emission:
490nm/525nm
Size:
100ul
Concentration:
1ug/ul
Applications:
WB(1:300-5000)
FCM(1:20-100)
IF(IHC-P)(1:50-200)
IF(IHC-F)(1:50-200)
IF(ICC)(1:50-200)
45
Human
Mouse
Rat
Predicted Cross Reactive Species:
Dog
Cow
Pig
Rabbit
For research use only. Not intended for diagnostic or therapeutic use.
PRODUCT SPECIFIC PUBLICATIONS
- Yutao Cui. et al. Functionalized Decellularized Bone Matrix Promotes Bone Regeneration by Releasing Osteogenic Peptides. ACS BIOMATER-SCI ENG. 2023;XXXX(XXX):XXX-XXXRead more>>
VALIDATION IMAGES
A549 cells(black) were fixed with 4% PFA for 10min at room temperature,permeabilized with PBST for 20 min at room temperature, and incubated in 5% BSA blocking buffer for 30 min at room temperature. Cells were then stained with Galactosidase alpha Polyclonal Antibody(bs-7593R-AF488)at 1:50 dilution in blocking buffer and incubated for 30 min at room temperature, washed twice with 2%BSA in PBS. Acquisitions of 20,000 events were performed. Cells stained with primary antibody (green), and isotype control (orange).