bs-7593R

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: 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Storage: Shipped at 4°C. Store at -20°C for one year. 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.

Size: 100ul

Concentration: 1ug/ul

Applications: WB(1:300-5000)
ELISA(1:500-1000)
FCM(1:20-100)
IHC-P(1:200-400)
IHC-F(1:100-500)
IF(IHC-P)(1:50-200)
IF(IHC-F)(1:50-200)
IF(ICC)(1:50-200)

Predicted Molecular Weight: 45

Cross Reactive Species: Human
Mouse
Rat

Predicted Cross Reactive Species: Dog
Cow
Pig
Rabbit

For research use only. Not intended for diagnostic or therapeutic use.