| Overview |
| bs-7593R-Biotin |
| Galactosidase alpha Polyclonal Antibody, Biotin Conjugated |
| WB, ELISA, IHC-P, IHC-F |
| Human, Mouse, Rat |
| Dog, Cow, Pig, Rabbit |
| Specifications |
| Biotin |
| Rabbit |
| KLH conjugated synthetic peptide derived from human Galactosidase alpha |
| 101-200/429 |
| Polyclonal |
| IgG |
| 1ug/ul |
| Purified by Protein A. |
| Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| Store at -20°C for 12 months. |
| Target |
| 2717 |
| P06280 |
| Cytoplasm |
| GALA; Alpha-galactosidase A; Alpha-D-galactosidase A; Alpha-D-galactoside galactohydrolase; Melibiase; GLA |
| 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. |
| Application Dilution |
| WB |
1:300-5000 |
| ELISA |
1:500-1000 |
| IHC-P |
1:200-400 |
| IHC-F |
1:100-500 |
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