- Antibody type:Primary
- Antigen name:Galactosidase Alpha
- Antigen symbol:GLA
- Clonality:Polyclonal
- Conjugation:Cy3®
- Host:Rabbit
- ImmunoChemistry:Yes
- Isotype:IgG
- Reactivity:Human,Rat,Mouse
- Environmentally Preferable:
- Cross adsorption:No
- Form:liquid
- Gene ID:2717
- Antigen synonyms:GLA|Alpha-galactosidase A|Alpha-D-galactoside galactohydrolase|Alpha-D-galactosidase A|GALA|Melibiase|Agalsidase
- Storage buffer:Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at 4°C for 12 months.
- Storage temperature:Store at –20 °C for 12 months
- Concentration:1 μg/μl
- Shipping temperature:4°C
- Immunogen:127-148/429
- Purification:Purified by Protein A
- Pk:100 µl
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.
Recommended Dilutions: IF(IHC-P): 1:50-200
Type: Primary
Antigen: Galactosidase alpha
Clonality: Polyclonal
Clone:
Conjugation: Cy3®
Epitope:
Host: Rabbit
Isotype: IgG
Reactivity: Human, Mouse, Rat