SMN1 抗体
Our Local Distributor
北京 101111
Quick Overview for SMN1 抗体 (ABIN7270651)
抗原
See all SMN1 抗体适用
宿主
克隆类型
标记
应用范围
-
-
原理
- SMN1 Rabbit mAb
-
交叉反应
- 人, 大鼠
-
产品特性
- Monoclonal Antibodies
-
纯化方法
- Affinity purification
-
免疫原
- A synthesized peptide derived from human SMN1
-
亚型
- IgG
-
-
-
-
应用备注
- WB,1:500 - 1:2000,IHC,1:50 - 1:200
-
限制
- 仅限研究用
-
-
-
状态
- Liquid
-
缓冲液
- PBS with 0.02 % sodium azide,0.05 % BSA,50 % glycerol, pH 7.3.
-
储存液
- Sodium azide
-
注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
-
储存条件
- -20 °C
-
储存方法
- Store at -20°C. Avoid freeze / thaw cycles.
-
-
- SMN1 (Survival of Motor Neuron 1, Telomeric (SMN1))
-
别名
- SMN1
-
背景
- This gene is part of a 500 kb inverted duplication on chromosome 5q13. 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. 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.,BCD541, GEMIN1, SMA, SMA1, SMA2, SMA3, SMA4, SMA@, SMN, SMNT, T-BCD541, TDRD16A,SMN1
-
分子量
- 36kDa
-
基因ID
- 6606
-
UniProt
- Q16637
-
途径
- Ribonucleoprotein Complex Subunit Organization
抗原
-