SMN1 polyclonal antibody
产品名称： SMN1 polyclonal antibody
英文名称： SMN1 polyclonal antibody
产品编号： PAB12734
产品价格： null
产品产地： 台湾
品牌商标： Abnova
更新时间： null
使用范围：
亚诺法生技股份有限公司（Abnova)
- 联系人 :
- 地址 : 台湾台北市内湖区洲子街 108 号 9 楼
- 邮编 : 11493
- 所在区域 : 台湾
- 电话 : +886-920**1152 点击查看
- 传真 : 点击查看
- 邮箱 : sales@abnova.com.tw
- Specification
- Product Description:
- Rabbit polyclonal antibody raised against synthetic peptide of SMN1.
- Immunogen:
- A synthetic peptide corresponding to C-terminus of human SMN1.
- Host:
- Rabbit
- Theoretical MW (kDa):
- 35
- Reactivity:
- Human
- Specificity:
- This antibody recognizes ~35 KDa of human SMN1.
- Form:
- Liquid
- Storage Buffer:
- In PBS, pH 7.2 (10% Proclin300)
- Storage Instruction:
- Store at 4°C. For long term storage store at -20°C or lower.
Aliquot to avoid repeated freezing and thawing.
- Quality Control Testing:
- Antibody Reactive Against Synthetic Peptide.
- Recommend Usage:
- Western Blot (0.1-1 ug/mL)
ELISA (0.01-0.1 ug/mL)
Immunoprecipitation (2-5 ug/mL)
The optimal working dilution should be determined by the end user.
- Publication Reference
- 1.
- Gemin2 plays an important role in stabilizing the survival of motor neuron complex.
Ogawa C, Usui K, Aoki M, Ito F, Itoh M, Kai C, Kanamori-Katayama M, Hayashizaki Y, Suzuki H.J Biol Chem. 2007 Apr 13;282(15):11122-34. Epub 2007 Feb 16.
- Entrez GeneID:
- 6606
- Gene Name:
- SMN1
- Gene Alias:
- BCD541,SMA,SMA1,SMA2,SMA3,SMA4,SMA@,SMN,SMNT,T-BCD541
- Gene Description:
- survival of motor neuron 1, telomeric
- Gene Ontology:
- Hyperlink
- Gene Summary:
- 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. Two transcript variants encoding distinct isoforms have been described. [provided by RefSeq
- Other Designations:
- OTTHUMP00000125198,gemin 1