-
SMN1 antibody - N-terminal region
SMN1 antibody - N-terminal region
- 中文名称：
- SMN1 antibody - N-terminal region
- 英文名称：
- SMN1 antibody - N-terminal region
- 品牌：
- AAA Biotech
- 品牌介绍：
- AAA Biotech专注于为全球生命科学研究提供高品质的蛋白质研究工具，核心产品包括经严格验证的抗体、重组蛋白及ELISA试剂盒。
- 货号：
- AAA198588
- 规格：
- 0.1 mL|2x0.1 mL|3x0.1 mL|4x0.1 mL|5x0.1 mL
- 保存建议：
- 短期使用时，在2-8摄氏度下储存一周。对于长期储存，以小份储存在-20摄氏度，以防止冻融循环。
- 货期：
- 6-8周
- 来源宿主：
- Rabbit
- 反应种属：
- Dog, Guinea Pig, Horse, Human, Mouse, Pig, Rabbit, Rat
- 应用：
- WB (Western Blot)
- 免责声明：
- *本产品仅供科研实验使用，不得用于临床诊断。*
- 其他：
克隆性:Polyclonal
同型:N/A
克隆号:N/A
特异性:N/A
纯度:Affinity Purified
形式：Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
浓度：N/A
- 说明书：
This is a rabbit polyclonal antibody against SMN1. It was validated on Western Blot using a cell lysate as a positive control.
Target Description: SMN1 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.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. 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 are produced by this gene.
