EK1260

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

X

¥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

¥<\/span>1,600.00<\/bdi><\/span><\/span>","sku":"70-EK1260-48","variation_description":"","variation_id":10062,"variation_is_active":true,"variation_is_visible":true,"weight":"","weight_html":"N\/A"},{"attributes":{"attribute_size":"96T"},"availability_html":"","backorders_allowed":false,"dimensions":{"length":"","width":"","height":""},"dimensions_html":"N\/A","display_price":2650,"display_regular_price":2650,"image":{"title":"ELISA-1.jpg","caption":"","url":"https:\/\/www.liankebio.com\/wp-content\/uploads\/ELISA-1.jpg","alt":"","src":"https:\/\/www.liankebio.com\/wp-content\/uploads\/ELISA-1.jpg","srcset":"https:\/\/www.liankebio.com\/wp-content\/uploads\/ELISA-1.jpg 800w, https:\/\/www.liankebio.com\/wp-content\/uploads\/ELISA-1-768x576.jpg 768w","sizes":"(max-width: 800px) 100vw, 800px","full_src":"https:\/\/www.liankebio.com\/wp-content\/uploads\/ELISA-1.jpg","full_src_w":800,"full_src_h":600,"gallery_thumbnail_src":"https:\/\/www.liankebio.com\/wp-content\/uploads\/ELISA-1-100x100.jpg","gallery_thumbnail_src_w":100,"gallery_thumbnail_src_h":100,"thumb_src":"https:\/\/www.liankebio.com\/wp-content\/uploads\/ELISA-1.jpg","thumb_src_w":800,"thumb_src_h":600,"src_w":800,"src_h":600},"image_id":5986,"is_downloadable":false,"is_in_stock":true,"is_purchasable":true,"is_sold_individually":"no","is_virtual":false,"max_qty":"","min_qty":1,"price_html":"¥<\/span>2,650.00<\/bdi><\/span><\/span>","sku":"70-EK1260-96","variation_description":"","variation_id":10063,"variation_is_active":true,"variation_is_visible":true,"weight":"","weight_html":"N\/A"}]">

描述

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.

操作步骤


下载本产品说明书

ELISA操作常见问题

查看更多ELISA操作相关问题

引用文献


评价

目前还没有评价

成为第一个“Human alpha-Galactosidase A/GLA ELISA Kit检测试剂盒(酶联免疫吸附法)” 的评价者

您的电子邮箱地址不会被公开。 必填项已用*标注