半岛bd体育手机客户端 说明
免疫原
Synthetic peptide directed towards the N terminal region of human SMN1
生化/生理作用
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.
序列
Synthetic peptide located within the following region: KAVASFKHALKNGDICETSGKPKTTPKRKPAKKNKSQKKNTAASLQQWKV
外形
Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
免责声明
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
基本信息
NACRES | NA.41 |
半岛bd体育手机客户端 性质
生物来源 | rabbit |
质量水平 | 100 |
偶联物 | unconjugated |
抗体形式 | affinity isolated antibody |
antibody product type | primary antibodies |
克隆 | polyclonal |
形式 | buffered aqueous solution |
分子量 | 28kDa |
species reactivity | dog, mouse, human |
浓度 | 0.5 mg - 1 mg/mL |
technique(s) | immunoblotting: suitable |
NCBI登记号 | NM_022874 |
UniProt登记号 | Q16637 |
运输 | wet ice |
储存温度 | ?20℃ |
Gene Information | human ... SMN1(6606) |
安全信息
储存分类代码 | 12 - Non Combustible Liquids |
WGK | WGK 3 |
闪点(F) | Not applicable |
闪点(C) | Not applicable |
Sigma-Aldrich