
产品货号:
Z34190
中文名称:
SMN1/2抗体
英文名称:
Anti-SMN1/2 Antibody(Clone#2F1)
产品规格:
100μl
发货周期:
1~3天
产品价格:
询价
抗体名称 | Anti-SMN1/2 Antibody (Clone#2F1) |
指标别名 | C BCD541;Component of gems 1;Gemin 1;SMN;SMN1;SMN1;SMN;SMN2;SMNC;SMNT;Survival motor neuron protein |
克隆性 | Monoclonal |
检验物种 | human,monkey |
应用范围 | WB,IHC,FCM,ELISA |
基因名称 | SMN1/SMN2 |
克隆号 | 2F1 |
抗体来源 | Mouse |
抗体类型 | IgG |
免疫原 | Purified recombinant fragment of human SMN1 expressed in E.Coli. |
成分 | Ascitic fluid containing 0.03% sodium azide. |
纯化方式 | Affinity purification |
浓度 | 1mg/mL |
产品形态 | 溶液 |
保存条件 | -20℃可保存1年,短期保存或频繁使用请置于4℃,可保存一个月,避免反复冻融。 |
背景资料 | This gene is part of a 500kb 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. |
Uniprot ID | Q16637/Q16637 |
推荐稀释比 | WB 1:500~1:2000 IHC 1:200~1:1000 FC 1:200~1:400 ELISA 1:10000 |
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