| 產(chǎn)品編號(hào) | bs-7593R-BF594 |
| 英文名稱 | Rabbit Anti-Galactosidase alpha/BF594 Conjugated antibody |
| 中文名稱 | BF594標(biāo)記的α-半乳糖苷酶抗體 |
| 別 名 | Galactosidase alpha; Alpha D galactosidase A; Alpha D galactoside galactohydrolase; Melibiase; Alpha galactosidase A; GALA; GLA; GLA protein; AGAL_HUMAN. |
| 規(guī)格價(jià)格 | 100ul/2980元 購(gòu)買 大包裝/詢價(jià) |
| 說(shuō) 明 書 | 100ul |
| 研究領(lǐng)域 | 心血管 細(xì)胞生物 免疫學(xué) 神經(jīng)生物學(xué) |
| 抗體來(lái)源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | |
| 產(chǎn)品應(yīng)用 | ICC=1:50-200 IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 45kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Galactosidase alpha |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲(chǔ) 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 產(chǎn)品介紹 |
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. Subunit: Homodimer. Subcellular Location: Lysosome. DISEASE: Defects in GLA are the cause of Fabry disease (FD) [MIM:301500]. FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. 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. Similarity: Belongs to the glycosyl hydrolase 27 family. Database links: Entrez Gene: 2717 Human Entrez Gene: 11605 Mouse Omim: 300644 Human SwissProt: P06280 Human SwissProt: P51569 Mouse Unigene: 69089 Human Unigene: 1114 Mouse
Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
