FITC标记的α葡萄糖苷酶/溶酶体α-葡糖苷酶抗体
产品名称： FITC标记的α葡萄糖苷酶/溶酶体α-葡糖苷酶抗体
英文名称： Anti-GAA/FITC
产品编号： HZ-13254R-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： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-GAA/FITC Conjugated antibody
FITC标记的α葡萄糖苷酶/溶酶体α-葡糖苷酶抗体
| 英文名称 | Anti-GAA/FITC |
| 中文名称 | FITC标记的α葡萄糖苷酶/溶酶体α-葡糖苷酶抗体 |
| 别 名 | 70 kDa lysosomal alpha-glucosidase; Acid alpha glucosidase; Acid maltase; Aglucosidase alfa; Alpha glucosidase; GAA; Glucosidase alpha acid (Pompe disease glycogen storage disease type II); Glucosidase alpha acid; Glucosidase alpha; LYAG; LYAG_HUMAN; Lysosomal alpha glucosidase. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 细胞生物 信号转导 新陈代谢 表观遗传学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Pig, Horse, Sheep, |
| 产品应用 | 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 | 83/92/98kDa |
| 细胞定位 | 细胞膜 |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human GAA/Glucosidase 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: This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Jul 2008]. Function: Essential for the degradation of glygogen to glucose in lysosomes. Subcellular Location: Lysosome. Lysosome membrane. Post-translational modifications: The different forms of acid glucosidase are obtained by proteolytic processing. Phosphorylation of mannose residues ensures efficient transport of the enzyme to the lysosomes via the mannose 6-phosphate receptor. DISEASE: Defects in GAA are the cause of glycogen storage disease type 2 (GSD2) [MIM:232300]; also called acid alpha-glucosidase (GAA) deficiency or acid maltase deficiency (AMD). GSD2 is a metabolic disorder with a broad clinical spectrum. The severe infantile form, or Pompe disease, presents at birth with massive accumulation of glycogen in muscle, heart and liver. Cardiomyopathy and muscular hypotonia are the cardinal features of this form whose life expectancy is less than two years. The juvenile and adult forms present as limb-girdle muscular dystrophy beginning in the lower limbs. Final outcome depends on respiratory muscle failure. Patients with the adult form can be free of clinical symptoms for most of their life but finally develop a slowly progressive myopathy. Similarity: Belongs to the glycosyl hydrolase 31 family. Contains 1 P-type (trefoil) domain. Database links: Entrez Gene: 2548 Human Entrez Gene: 14387 Mouse Entrez Gene: 367562 Rat Omim: 606800 Human SwissProt: P10253 Human SwissProt: P70699 Mouse SwissProt: Q6P7A9 Rat Unigene: 1437 Human Unigene: 4793 Mouse Unigene: 162368 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
酸性α-葡萄糖苷酶基因encodes this，which is to for the of glycogen基本lysosomes葡萄糖降解。酸性α-葡萄糖苷酶forms of different are obtained proteolytic处理模式。在这个基因缺陷的原因是存储glycogen II of known as pompe'也病，阿尔茨海默病，which is an autosomal隐性疾病与广泛的临床频谱。三成绩单variants encoding the same for this have been found蛋白基因。[提供]序列模式，2008年7月。