推荐产品
生物来源
mouse
质量水平
偶联物
unconjugated
抗体形式
ascites fluid
抗体产品类型
primary antibodies
克隆
2F1, monoclonal
分子量
39 kDa
种属反应性
monkey, human
技术
direct ELISA: 1:10,000
flow cytometry: 1:200-1:400
immunohistochemistry: 1:200-1:1,000
western blot: 1:500-1:2,000
同位素/亚型
IgG1
NCBI登记号
运输
wet ice
储存温度
−20°C
靶向翻译后修饰
unmodified
基因信息
human ... SMN1(6606)
一般描述
Survival motor neuron 1 (SMN1), also known as gemin 1, is a survival motor neuron protein, which is highly expressed in spinal cord. SMN1 is localized on the telomeric region of human chromosome 5q13. The 38kDA protein is expressed mostly in nucleus and cytoplasm.
免疫原
Purified recombinant fragment of human SMN1 expressed in E.coli.
Mouse monoclonal antibody raised against SMN1
Mouse monoclonal antibody raised against SMN1
生化/生理作用
Survival motor neuron 1 (SMN1) is involved in the synthesis of full length survival motor neuron (SMN) mRNA transcripts. SMN1 also contributes to the biogenesis and assembly of snRNP (small nuclear riboproteins) in the cytoplasm, which plays an important role in pre-mRNA splicing or processing. Axonal SMN protein mediates the growth of axon and mRNA axonal transport. Mutations in the SMN1 gene has been associated with a genetic neurodegenerative disease called spinal muscular atrophy(SMA).
外形
Ascitic fluid containing 0.03% sodium azide.
免责声明
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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储存分类代码
10 - Combustible liquids
WGK
WGK 3
闪点(°F)
Not applicable
闪点(°C)
Not applicable
法规信息
常规特殊物品
SMN affects membrane remodelling and anchoring of the protein synthesis machinery
Francesca G, et al.
Journal of Cell Science, jcs-176750 (2016)
Different stability and proteasome-mediated degradation rate of SMN protein isoforms
Locatelli D, et al.
PLoS ONE, 10(7), e0134163-e0134163 (2015)
SMN regulation in SMA and in response to stress: new paradigms and therapeutic possibilities
Dominguez CE, et al.
Human Genetics, 136(9), 1173-1191 (2017)
How do SMA-linked mutations of SMN1 lead to structural/functional deficiency of the SMA protein?
Li W
PLoS ONE, 12(6), e0178519-e0178519 (2017)
Identification and characterization of a spinal muscular atrophy-determining gene
Lefebvre S, et al.
Cell, 80(1), 155-165 (1995)
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