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重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 H WB, ELISA Ch Affinity Purified Polyclonal Antibody 描述 产品目录编号 AB3533 品牌系列 Chemicon®
商名 - Chemicon
描述 Anti-α Galactosidase Antibody 产品信息 格式 Affinity Purified 演示 Affinity purified immunoglobulin. Liquid in PBS containing 0.02% sodium azide. 应用 应用 Anti-α Galactosidase Antibody detects level of α Galactosidase & has been published & validated for use in ELISA & WB. 主要应用 - Western Blotting
- ELISA
应用说明 Immunoblotting: 1:1,000-1:5,000
ELISA: 1:1,000-1:5,000
Optimal working dilutions must be determined by the end user.生物信息 免疫原品种 Synthetic peptides from human galactosidase, alpha. 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Chicken 特异性 Galactosidase, alpha. 品种反应性 Human 抗体类型 Polyclonal Antibody Entrez基因编号 - NM_000169.2
Entrez基因汇总 This gene encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. This enzyme predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties. 基因符号 - EC 3.2.1.22
- GLA
- GALA
- melibiase
- Melibiase
UniProt编号 - P06280
UniProt汇总 SIZE: 429 amino acids; 48767 Da
SUBUNIT: Homodimer.
SUBCELLULAR LOCATION: Lysosome.
DISEASE: SwissProt: P06280 # 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: SwissProt: P06280 ## Belongs to the glycosyl hydrolase 27 family.产品使用声明 使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Maintain at -20°C in undiluted aliquots for up to 6 months after date of receipt. Avoid repeated freeze/thaw cycles. 包装信息 数量 100 µg
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