BD-PE0099-GBE1 (9R17) Mouse Monoclonal antibody-现货抗体产品库武汉枢密脑科学技术有限公司

GBE1 (9R17) Mouse Monoclonal antibody

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产品基本信息

产品货号

BD-PE0099

产品名称

GBE1 (9R17) Mouse Monoclonal antibody

类别

常规抗体

基因名称

GBE1

推荐应用

反应种属

Human,Mouse,Rat

存储缓冲液

PBS (pH 7.3) containing 1% BSA, 50% glycerol and 0.02% New type preservative N.

免疫原

Full length human recombinant protein of human GBE1 (NP_000149) produced in HEK293T cell.

稀释度

WB 1:2000, IF 1:100, FCM 1:100, IP 2ug/500ul

预测分子量

80.3kDa

运输及保存条件

-20°C/1 year

宿主

Mouse

同种型

IgG2a

背景介绍

The protein encoded by this gene is a glycogen branching enzyme that catalyzes the transfer of alpha-1,4-linked glucosyl units from the outer end of a glycogen chain to an alpha-1,6 position on the same or a neighboring glycogen chain. Branching of the chains is essential to increase the solubility of the glycogen molecule and, consequently, in reducing the osmotic pressure within cells. Highest level of this enzyme are found in liver and muscle. Mutations in this gene are associated with glycogen storage disease IV (also known as Andersen's disease). [provided by RefSeq]

期货

现货

纯化

Purified from mouse ascites fluids or tissue culture supernatant by affinity chromatography (protein A/G)

Flow cytometric Analysis of Hela cells, using anti-GBE1 antibody (BD-PE0099), (Red), compared to a nonspecific negative control antibody, (Blue).

Figure from citation: WB, performed with anti-GBE1 antibody (GBE1 mousemonoclonal antibody BD-PE0099, Origene), showed a decrease of protein on skeletal muscle of patients II-5 /II-6, being protein absent on cardiac muscle of a neonateaffected with the congenital variant of glycogenosis IV (GSD-IV or Andersen disease).

Anti-GBE1 mouse monoclonal antibody (BD-PE0099) immunofluorescent staining of COS7 cells transiently transfected by pCMV6-ENTRY GBE1 .

HEK293T cells were transfected with the pCMV6-ENTRY control (Left lane) or pCMV6-ENTRY GBE1 (Right lane) cDNA for 48 hrs and lysed. Equivalent amounts of cell lysates (5 ug per lane) were separated by SDS-PAGE and immunoblotted with anti-GBE1. Positive lysates (100ug) and (20ug) can be purchased separately from OriGene.