biological source
mouse
conjugate
unconjugated
antibody form
ascites fluid
antibody product type
primary antibodies
clone
2F1, monoclonal
mol wt
39 kDa
species reactivity
monkey, human
technique(s)
direct ELISA: 1:10,000, flow cytometry: 1:200-1:400, immunohistochemistry: 1:200-1:1,000, western blot: 1:500-1:2,000
isotype
IgG1
NCBI accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Quality Level
Gene Information
human ... SMN1(6606)
General description
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.
Immunogen
Purified recombinant fragment of human SMN1 expressed in E.coli.
Mouse monoclonal antibody raised against SMN1
Mouse monoclonal antibody raised against SMN1
Biochem/physiol Actions
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).
Physical form
Ascitic fluid containing 0.03% sodium azide.
Disclaimer
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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存储类别
10 - Combustible liquids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
法规信息
动植物来源生物产品
常规特殊物品
此项目有
SMN affects membrane remodelling and anchoring of the protein synthesis machinery
Francesca G, et al.
Journal of Cell Science, jcs-176750 (2016)
Identification and characterization of a spinal muscular atrophy-determining gene
Lefebvre S, et al.
Cell, 80(1), 155-165 (1995)
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)
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)
Different stability and proteasome-mediated degradation rate of SMN protein isoforms
Locatelli D, et al.
PLoS ONE, 10(7), e0134163-e0134163 (2015)
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