运动神经元生存蛋白1

SMN1 Antibody

Cat#：PL0302337

Isotype:Rabbit Polyclonal IgG

Immunogen：Peptide derived from human SMN1.

Purification: Immunogen affinity purified

Storage Buffer: PBS, pH 7.4(1%BSA and 0.1% Sodium azide)

Background：

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).

Specificity Reaction：Hu, Mo, Rat, Dog

Application Notes:WB: 1:100-500 E: 1:500-1000 IP: 1:20-100 IHC: 1:100-500 IF: 1:100-500

Storage ：

Store at –20ºC for one year. Avoid repeated freeze-thaw cycles. At 4ºC for 2-3 weeks after reconstituted.

This product for research use only, not for use in humans, therapeutic or diagnostic procedures.

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