生物来源
rabbit
质量水平
物料
colorless
克隆
9N9Q6, monoclonal
形式
liquid
分子量
38 kDa
种属反应性
rat, human
浓度
1.8 mg/mL
技术
immunohistochemistry: 1:50 - 1:200
western blot: 1:500 - 1:2000
颜色
colorless
同位素/亚型
IgG
免疫原序列
SEDGCIYPATIASIDFKRETCVVVYTGYGNREEQNLSDLLSPICEVANNIEQNAQENENESQVSTDESENSRSPGNKSDNIKPKSAPWNSFLPPPPPM
UniProt登记号
运输
wet ice
储存温度
−20°C
靶向翻译后修饰
unmodified
基因信息
human ... SMN1(6606)
一般描述
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.
免疫原
PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3.
Recombinant fusion protein containing a sequence corresponding to amino acids 103-200 of human SMN1 (Q16637).
应用
WB, IHC
WGK
WGK 1
法规信息
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