SMN1 Monoclonal Antibody

产品详情

产品名称

SMN1 Monoclonal Antibody

产品货号

YM-A04718

规格

50μL;100μL

种属反应

Human,Monkey

实验应用

WB,IHC,IF,ELISA

分子量

宿主

Mouse

同种型

修饰

Unmodified

推荐稀释比

WB 1:500-1:2000;IHC 1:200-1:1000;IF 1:200-1:1000;ELISA 1:10000;Not yet tested in other applications.

组成

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

纯化工艺

Affinity purification

储存

-15°C to -25°C/1 year(Do not lower than -25°C)

浓度

克降性

Monoclonal

克隆号

免疫原

Purified recombinant fragment of human SMN1 expressed in E. Coli.

特异性

SMN1 Monoclonal Antibody detects endogenous levels of SMN1 protein.

基因名称

SMN1

蛋白名称

Survival motor neuron protein

别名

SMN1;SMN;SMNT;SMN2;SMNC;Survival motor neuron protein;Component of gems 1;Gemin-1

基因ID-1

6606

SwissProt

Q16637

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 des

细胞定位

Nucleus, gem . Nucleus, Cajal body . Cytoplasm . Cytoplasmic granule . Perikaryon . Cell projection, neuron projection . Cell projection, axon . Cytoplasm, myofibril, sarcomere, Z line . Colocalizes with actin and at the Z-line of skeletal muscle (By similarity). Under stress conditions colocalizes with RPP20/POP7 in punctuated cytoplasmic granules (PubMed:14715275). Colocalized and redistributed with ZPR1 from the cytoplasm to nuclear gems (Gemini of coiled bodies) and Cajal bodies (PubMed:11283611). Colocalizes with FMR1 in cytoplasmic granules in the soma and neurite cell processes (PubMed:18093976). .