| 克隆類型 ： |
Rabbit Polyclonal IgG |
|
免 疫 原 ： |
Peptide derived from human SMN1. |
|
純化方式 ： |
Immunogen affinity purified |
|
緩 沖 液 ： |
PBS, pH 7.4(1%BSA and 0.1% Sodium azide) |
|
產(chǎn)品描述 ： |
SMN1 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. While mutations in the telomeric copy are associated with spinal muscular atrophy, mutations in this gene, the centromeric copy, do not lead to disease. This gene 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 full length 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). |
| 適用物種 ： |
Hu, Mo, Rat, Dog |
| 應(yīng)用說明 ： |
WB: 1:200-1:1000 E: 1:500-1:1000 IHC: 1:200-1:1000 IF: 1:100-1:500 |
| 別 名 ： |
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. |
| 保存條件 ： |
Store at -20°C for one year. Avoid repeated freeze-thaw cycles. At 4°C for 2-3 weeks after reconstituted. |
| 標(biāo) 記 物 ： |
PLLABS公司可供應(yīng)生物素、辣根過氧化物酶、熒光素標(biāo)記抗體 |
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產(chǎn)品說明 ： |
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