ARG64148
anti-SMN / Gemin1 antibody
anti-SMN / Gemin1 antibody for Western blot and Human
Gene Regulation antibody
概述
| 产品描述 | Goat Polyclonal antibody recognizes SMN / Gemin1 |
|---|---|
| 反应物种 | Hu |
| 应用 | WB |
| 特异性 | This antibody is expected to recognise isoforms b and d of SMN1 (NP_075012.1 and NP_000335.1) and all reported isoforms of SMN2 (NP_075013.1, NP_075014.1, NP_075015.1 and NP_059107.1). |
| 宿主 | Goat |
| 克隆 | Polyclonal |
| 同位型 | IgG |
| 靶点名称 | SMN / Gemin1 |
| 抗原物种 | Human |
| 抗原 | C-DESENSRSPGNKSDN |
| 偶联标记 | Un-conjugated |
| 別名 | SMA3; SMA2; SMA1; Component of gems 1; SMNT; SMA4; GEMIN1; Survival motor neuron protein; BCD541; SMN; SMA@; T-BCD541; TDRD16A; SMA; Gemin-1 |
应用说明
| 应用建议 |
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|---|---|---|---|---|---|
| 应用说明 | WB: Recommend incubate at RT for 1h. * The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist. |
属性
| 形式 | Liquid |
|---|---|
| 纯化 | Purified from goat serum by antigen affinity chromatography. |
| 缓冲液 | Tris saline (pH 7.3), 0.02% Sodium azide and 0.5% BSA. |
| 抗菌剂 | 0.02% Sodium azide |
| 稳定剂 | 0.5% BSA |
| 浓度 | 0.5 mg/ml |
| 存放说明 | For continuous use, store undiluted antibody at 2-8°C for up to a week. For long-term storage, aliquot and store at -20°C or below. Storage in frost free freezers is not recommended. Avoid repeated freeze/thaw cycles. Suggest spin the vial prior to opening. The antibody solution should be gently mixed before use. |
| 注意事项 | For laboratory research only, not for drug, diagnostic or other use. |
生物信息
| 数据库连接 | |
|---|---|
| 基因名称 | SMN1 |
| 全名 | survival of motor neuron 1, telomeric |
| 背景介绍 | 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. Multiple transcript variants encoding distinct isoforms have been described. [provided by RefSeq, Jul 2014] |
| 生物功能 | The SMN complex plays a catalyst role in the assembly of small nuclear ribonucleoproteins (snRNPs), the building blocks of the spliceosome. Thereby, plays an important role in the splicing of cellular pre-mRNAs. Most spliceosomal snRNPs contain a common set of Sm proteins SNRPB, SNRPD1, SNRPD2, SNRPD3, SNRPE, SNRPF and SNRPG that assemble in a heptameric protein ring on the Sm site of the small nuclear RNA to form the core snRNP. In the cytosol, the Sm proteins SNRPD1, SNRPD2, SNRPE, SNRPF and SNRPG are trapped in an inactive 6S pICln-Sm complex by the chaperone CLNS1A that controls the assembly of the core snRNP. Dissociation by the SMN complex of CLNS1A from the trapped Sm proteins and their transfer to an SMN-Sm complex triggers the assembly of core snRNPs and their transport to the nucleus. Ensures the correct splicing of U12 intron-containing genes that may be important for normal motor and proprioceptive neurons development. May also play a role in the metabolism of small nucleolar ribonucleoprotein (snoRNPs). [UniProt] |
| 研究领域 | Gene Regulation antibody |
| 预测分子量 | 32 kDa |
检测图片 (1) Click the Picture to Zoom In
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ARG64148 anti-SMN / Gemin1 antibody WB image
Western blot: 35 µg of Human cerebellum lysate (in RIPA buffer) stained with ARG64148 anti-SMN / Gemin1 antibody at 1 µg/ml dilution and incubated at RT for 1 hour.
