Quantity size:
0.2ml
Concentration:
1mg/ml 脊髓性肌萎缩症蛋白SMA/抗体 Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Background:
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 脊髓性肌萎缩症蛋白SMA/抗体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 脊髓性肌萎缩症蛋白SMA/抗体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, Sep 2008]
Also known as:
Component of gems 1; Component of gems 2; Gemin 1; Gemin-1; SMA; SMA1; SMA3; SMN; SMN_HUMAN; SMN1; SMN2; SMNC; SMNT; Survival motor neuron protein; survival of motor neuron 1, telomeric; survival of motor neuron 2, centromeric;Component of gems 1; Gemin 1; Gemin-1; Gemin1; SMA 1;脊髓性肌萎缩症蛋白SMA/抗体 SMA 2; SMA 3; SMA 4; SMA; SMA1; SMA2; SMA3; SMA4; SMN 1; SMN; SMN-1; SMN_HUMAN; SMN1; SMN2; SMNT; Survival motor neuron protein; Survival of motor neuron 1 (telomeric); survival of motor neuron 1; Survival of motor neuron 1, telomeric; T-BCD541; BCD541; SMN_HUMAN.
Specificity:
●
Rabbit Polyclonal IgG, affinity purified by Protein A.
●
Reacts with: Human, Mouse, Rat, Dog, Pig, Cow, Rabbit, .
●
Immunogen: KLH conjugated synthetic peptide derived from human Gemin 1.
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脊髓性肌萎缩症蛋白SMA/抗体Predicted Molecular Weight: 31kDa.
Storage:
Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).
Application:
WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500
Not yet tested in other applications.
Optimal working dilutions must be determined by the end user.
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企业名称
上海康朗生物科技有限公司
企业信息已认证
企业类型
信用代码
310112001510759
成立日期
2015-05-29
注册资本
100
经营范围
从事生物科技技术领域内技术开发、技术咨询、技术服务、技术转让,实验分析仪器、化工产品及原料(除危险化学品、监控化学品、烟花爆竹、民用爆竹物品、易制毒化学品)、仪器仪表、环保设备、酒店用品、皮革制品、床上用品、电子产品的销售。【依法须经批准的项目,经相关部门批准后方可开展经营活动】
上海康朗生物科技有限公司
公司地址
上海市闵行区闵北路88弄1-30号第22栋BV145室
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