深圳欣博盛生物科技有限公司 a1

SMN1 antibody [2F1]
货号:GTX60451 规格:100μl 目录价:¥5800
产品详情
* 以下信息仅供参考,详情请以原厂网站为准
产品名称:
SMN1 antibody [2F1]
别名:
survival of motor neuron 1, telomeric , BCD541 , GEMIN1 , SMA , SMA1 , SMA2 , SMA3 , SMA4 , SMA@ , SMN , SMNT , T-BCD541 , TDRD16A
反应种属:
Human, Monkey
宿主来源:
Mouse
实验应用:
ELISA, FCM, IHC-P, WB
同种型:
IgG1
免疫原:
Purified recombinant fragment(148-294 a.a.) of human SMN1 expressed in E. Coli.
克隆性:
Monoclonal
克隆号:
2F1
纯化方式:
Unpurified
偶联:
Unconjugated
产品浓度:
保存温度:
Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.
运输温度:
4°C
产品形式:
Liquid
存储溶液:
Ascites, 0.03% Sodium azide.
生产商:
GeneTex
功能与背景:
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 t
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