EK1260

Human alpha-Galactosidase A/GLA ELISA Kit检测试剂盒（酶联免疫吸附法）

¥1,600.00 – ¥2,650.00

分子靶点：GLA, GALA
种属：人 (Human)
样本类型：血清，血浆，细胞培养上清及其他生物学样本
检测样本体积：20 μL
灵敏度：53.55 pg/mL
检测范围：0.5 ng/mL - 32 ng/mL
回收率：73% - 127%

在售SKU:70-EK1260-48, 70-EK1260-96

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描述

文章目录[隐藏]

分子信息
GLA 分子靶点信息概述
GLA 分子靶点综述
- 人 Human GLA 分子靶点信息
- 人 Human GLA靶点分子功能（预测）

商品名	Human alpha-Galactosidase A/GLA ELISA Kit (人α-半乳糖苷酶A ELISA试剂盒)
检测方法	双抗夹心法
精密度	板内变异系数：4.9% - 8.5%；板间变异系数：3.5%-7.7%
样本类型	血清，血浆，细胞培养上清及其他生物学样本
检测样本体积	20 μL
灵敏度	53.55 pg/mL
检测范围	0.5 ng/mL - 32 ng/mL
回收率	73% - 127%
平均回收率	0.93
板式	96孔板，可拆
保存	试剂盒未拆开，4℃保存。已拆开，标准品-20℃保存，其它4℃保存。
运输条件	4℃蓝冰运输
组分	包被抗alpha-Galactosidase A/GLA 单克隆抗体的96孔聚苯乙烯酶标板人alpha-Galactosidase A/GLA 冻干标准品人alpha-Galactosidase A/GLA 检测抗体标准品稀释液检测缓冲液(10×) 底物(TMB) 终止液洗液(20×) 封板膜

检测原理：本试剂盒采用双抗体夹心酶联免疫吸附检测技术。特异性抗人GLA预包被在高亲和力的酶标板上。酶标板孔中加入标准品和待测样本，经过孵育，样本中存在的GLA与固相抗体结合。洗涤去除未结合物质后，加入辣根过氧化物酶标记的检测抗体孵育。洗涤去除未结合的检测抗体。加入显色底物TMB，避光显色。颜色反应的深浅与样本中GLA的浓度成正比。加入终止液终止反应，在450 nm波长 (参考波长570 - 630 nm)测定吸光度值。

Human alpha-Galactosidase A/GLA ELISA Kit (人α-半乳糖苷酶A ELISA试剂盒) - 标准曲线 — 标准曲线

分子信息

概述人 GLA, GALA 靶点信息

GLA 分子靶点信息概述

分子名：GLA, galactosidase alpha
基因家族：Galactosidases alpha
别名：GALA

GLA 分子靶点综述

α-半乳糖苷酶A，又名GLA，是糖基水解酶27家族的一个成员。GLA可作为已确诊法布里病(FD)患者的长期酶替代疗法。GLA缺乏引发FD，这是一种罕见的X连锁的鞘脂类代谢障碍疾病，表现为糖脂沉积在许多组织中。FD是一种先天的鞘糖脂分解代谢异常疾病，患有该病的患者整个机体表现出神经酰胺三己糖苷(Gb3)和相关糖鞘脂类全身性沉积于血浆和细胞溶酶体中。男性下躯干的特征性皮肤病变(血管角质瘤)是医生对FD的临床识别。另外，患者可能出现眼睛沉淀物、发热和四肢烧灼痛。肾功能衰竭、高血压引起的心脏或大脑并发症或者其他血管疾病都会导致FD患者死亡。缺乏GLA导致糖鞘脂类沉积在血管中，进而导致多器官发生病变。除了微血管疾病，缺乏GLA还会导致早期大血管病变如中风和心肌梗死。

人 Human GLA 分子靶点信息

分子名：GLA, galactosidase alpha
别称：
- agalsidase alfa
- alpha-D-galactosidase A
- alpha-D-galactoside galactohydrolase 1
- alpha-gal A
- alpha-galactosidase A
- GALA
- galactosidase, alpha
- galactosylgalactosylglucosylceramidase GLA
- melibiase
基因序列：NCBI_Gene: 2717
蛋白序列：UniProtKB: P06280

人 Human GLA靶点分子功能（预测）

Enables hydrolase activity, hydrolyzing O-glycosyl compounds; protein homodimerization activity; and signaling receptor binding activity. Involved in glycosphingolipid catabolic process and oligosaccharide metabolic process. Located in Golgi apparatus; extracellular region; and lysosome. Implicated in Fabry disease.