SMN-Exon7 Monoclonal antibody, PBS Only

SMN-Exon7 Monoclonal Antibody for WB, IHC, IF/ICC, Indirect ELISA
Cat No. 60255-1-PBS

产品说明书

CloneNo. 3A8G11

宿主/亚型

Mouse / IgG1

种属反应性

human, mouse, rat

应用

WB, IHC, IF/ICC, Indirect ELISA

SMN, SMA, Gemin-1, Gemin 1, Component of gems 1

缓冲液配方:  PBS Only
PBS and Azide
PBS Only
偶联物:  Unconjugated
规格: 

-/ -


产品信息

60255-1-PBS targets SMN-Exon7 in WB, IHC, IF/ICC, Indirect ELISA applications and shows reactivity with human, mouse, rat samples.

经测试应用 WB, IHC, IF/ICC, Indirect ELISA Application Description
经测试反应性 human, mouse, rat
免疫原 SMN-Exon7 fusion protein Ag16615 种属同源性预测
宿主/亚型 Mouse / IgG1
抗体类别 Monoclonal
产品类型 Antibody
全称 survival of motor neuron 1, telomeric
别名 SMN, SMA, Gemin-1, Gemin 1, Component of gems 1
计算分子量 294 aa, 32 kDa
观测分子量 40 kDa
GenBank蛋白编号BC062723
基因名称 SMN
Gene ID (NCBI) 6606
RRIDAB_2881376
偶联类型 Unconjugated
形式 Liquid
纯化方式Protein A purification
UNIPROT IDQ16637
储存缓冲液 PBS only , pH 7.3
储存条件Store at -80°C.
The product is shipped with ice packs. Upon receipt, store it immediately at -80°C

背景介绍

Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of anterior horn cells in the spinal cord and concomitant symmetrical muscle weakness and atrophy (PMID: 16364894 ). SMA is caused by deletion or mutations of the survival motor neuron (SMN1) gene. SMA patients lack a functional SMN1 gene, but they possess an intact SMN2 gene, which though nearly identical to SMN1, is only partially functional (PMID: 17355180). A large majority of SMN2 transcripts lack exon 7, resulting in production of a truncated, less stable SMN protein (PMID: 10369862). The level of SMN protein correlates with phenotypic severity of SMA. This antibody, 60255-1-Ig, raised against the C-terminal region (275-294aa) encoded by the exon 7.

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