- 产品信息
商品描述
靶点-Target
Gemin 1
反应性
Human,Mouse,Rat
应用
WB,IHC,IF,IP,ELISA
宿主物种-Host Species
Rabbit
同种型-Isotype
IgG,Kappa
修饰-Modification
Unmodified
MW(Calculated)
32kD
MW(Observed)
38kD
推荐稀释比
IHC 1:200-1:500;WB 1:1000-1:20000;IF 1:200-1:1000;ELISA 1:5000-1:20000;IP 1:50-1:200;
组成
PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
纯化工艺
Protein A
储存
-15°C to -25°C/1 year(Do not lower than -25°C)
克隆号
Monoclonal
免疫原
PT1495R
特异性
Endogenous
基因名称
SMN1;SMN2
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
Organism-1
Human
基因ID-1
6606
SwissProt-1
Q16637
Organism-2
Mouse
SwissProt-2
P97801
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). .





