MUbio

alpha-Glucosidase Monoclonal Antibody (43G7)

Name: alpha-Glucosidase Monoclonal Antibody (43G7)
Brand: MUbio
SKU: MUB0707P

查看其他15个GAA抗体

说明书

实验方案

问题与答案

技术支持

说明书

实验方案

问题与答案

技术支持

说明书

实验方案

问题与答案

技术支持

产品信息

MUB0707P

应用

建议稀释比

已发表文章

免疫印迹 (WB)

Assay-dependent

免疫组化（石蜡） (IHC (P))

Assay-dependent

产品规格

种属反应

Human

宿主/亚型

Mouse / IgG2a

分类

Monoclonal

类型

Antibody

克隆号

43G7

抗原

alpha-Glucosidase

偶联物

Unconjugated Unconjugated Unconjugated

形式

Liquid

浓度

1 mg/mL

纯化类型

Protein A/G

保存液

PBS

内含物

0.09% sodium azide

保存条件

Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.

运输条件

Ambient (domestic); Wet ice (international)

产品详细信息

reacts with the two major bands with apparent molecular weights of 76.000 and 70.000, the band with a molecular weight of about 94.000 and minor bands with apparent molecular weights of less than 67.000, when analyzing a-glucosidase isolated from placenta by polyacrylamide gel electrophoresis in the presence of SDS and a reducing agent.

The antibody may be stored at 4°C. For prolonged storage prepare appropriate aliquots and store at or below -20°C. Prior to use, an aliquot is thawed slowly in the dark at ambient temperature, spun down again and used to prepare working dilutions by adding sterile phosphate buffered saline (PBS, pH 7.2). Repeated thawing and freezing should be avoided. Working dilutions should be stored at 4°C, not refrozen, and preferably used the same day. If a slight precipitation occurs upon storage, this should be removed by centrifugation. It will not affect the performance or the concentration of the product.

靶标信息

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.

仅用于科研。不用于诊断过程。未经明确授权不得转售。