| 克隆類(lèi)型 ： |
Rabbit Polyclonal IgG |
|
免 疫 原 ： |
Peptide derived from Hu Galactosidase alpha. |
|
純化方式 ： |
Immunogen affinity purified |
|
緩 沖 液 ： |
PBS, pH 7.4(1%BSA and 0.1% Sodium azide) |
|
產(chǎn)品描述 ： |
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. |
| 適用物種 ： |
Hu, Mo, Rat, Dog, Pig, Cow |
| 應(yīng)用說(shuō)明 ： |
E: 1:500-1:1000 IHC: 1:200-1:1000 IF: 1:100-1:500 |
| 別 名 ： |
Galactosidase alpha; Alpha D galactosidase A; Alpha D galactoside galactohydrolase; Melibiase; Alpha galactosidase A; GALA; GLA; GLA protein; AGAL_Hu. |
| 保存條件 ： |
Store at -20°C for one year. Avoid repeated freeze-thaw cycles. At 4°C for 2-3 weeks after reconstituted. |
| 標(biāo) 記 物 ： |
PLLABS公司可供應(yīng)生物素、辣根過(guò)氧化物酶、熒光素標(biāo)記抗體 |
|
產(chǎn)品說(shuō)明 ： |
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