SMN1 antibody-多抗-深圳欣博盛生物科技有限公司

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SMN1 antibody

品牌：GeneTex

官网链接

说明书

折扣/货期

货号：GTX101047	规格：100μl	目录价：￥4000
货号：GTX101047-S	规格：25μl	目录价：￥1700

产品详情

* 以下信息仅供参考，详情请以原厂网站为准

产品名称:

SMN1 antibody

别名:

survival of motor neuron 1, telomeric , BCD541 , GEMIN1 , SMA , SMA1 , SMA2 , SMA3 , SMA4 , SMA@ , SMN , SMNT , T-BCD541 , TDRD16A

反应种属:

Human

宿主来源:

Rabbit

实验应用:

ICC/IF, WB

同种型:

IgG

免疫原:

Recombinant protein encompassing a sequence within the center region of human SMN1. The exact sequence is proprietary.

克隆性:

Polyclonal

克隆号:

纯化方式:

Purified by antigen-affinity chromatography.

偶联:

Unconjugated

产品浓度:

1 mg/ml (Please refer to the vial label for the specific concentration.)

保存温度:

Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.

运输温度:

4°C

预期分子量:

产品形式:

Liquid

存储溶液:

PBS, 40% Glycerol, 0.01% Thimerosal.

生产商:

GeneTex

功能与背景:

This gene is part of a 500 kb inverted duplication on chromosome 5q13. This duplicated region contains at least four genes and repetitive elements which make it prone to rearrangements and deletions. The repetitiveness and complexity of the sequence have also caused difficulty in determining the organization of this genomic region. The telomeric and centromeric copies of this gene are nearly identical and encode the same protein. However, mutations in this gene, the telomeric copy, are associated with spinal muscular atrophy; mutations in the centromeric copy do not lead to disease. The centromeric copy may be a modifier of disease caused by mutation in the telomeric copy. The critical sequence difference between the two genes is a single nucleotide in exon 7, which is thought to be an exon splice enhancer. Note that the nine exons of both the telomeric and centromeric copies are designated historically as exon 1, 2a, 2b, and 3-8. It is thought that gene conversion events may involve the two genes, leading to varying copy numbers of each gene. The protein encoded by this gene localizes to both the cytoplasm and the nucleus. Within the nucleus, the protein localizes to subnuclear bodies called gems which are found near coiled bodies containing high concentrations of small ribonucleoproteins (snRNPs). This protein forms heteromeric complexes with proteins such as SIP1 and GEMIN4, and also interacts with several proteins known to be involved in the biogenesis of snRNPs, such as hnRNP U protein and the small nucleolar RNA binding protein. Two transcript variants encoding distinct isoforms have been described. [provided by RefSeq]

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