FITC标记的α-半乳糖苷酶抗体
产品名称： FITC标记的α-半乳糖苷酶抗体
英文名称： Anti-Galactosidase alpha/FITC
产品编号： HZ-7593R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： IF=1:50-200
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Rabbit Anti-Galactosidase alpha/FITC Conjugated antibody
FITC标记的α-半乳糖苷酶抗体
| 英文名称 | Anti-Galactosidase alpha/FITC |
| 中文名称 | FITC标记的α-半乳糖苷酶抗体 |
| 别 名 | Galactosidase alpha; Alpha D galactosidase A; Alpha D galactoside galactohydrolase; Melibiase; Alpha galactosidase A; GALA; GLA; GLA protein; AGAL_HUMAN. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 心血管 细胞生物 免疫学 神经生物学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Pig, Cow, Rabbit, |
| 产品应用 | 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 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Galactosidase alpha |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | 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. |
| 产品介绍 | 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 Entrez Gene: 363494 Rat 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 |
半乳糖苷酶α参与了α-D半乳糖苷（包括半乳糖寡糖、半乳甘露聚糖和半乳糖水解酶）的非还原性α-D半乳糖残基的水解。GLA缺陷是Fabry病（FD）的病因。FD是一种罕见的X-连锁鞘脂贮积病，糖脂在许多组织中积累。男性的临床识别是由下躯干的特征性皮肤病变（血管角化囊肿）引起的。患者可能出现眼部沉积物、发热性发作和四肢灼痛。死亡是由肾衰竭、高血压或其他血管疾病的心脏或脑并发症引起的。杂合性女性可能表现为弱形式的紊乱，更容易表现出COR。