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SAB5300203

Sigma-Aldrich

Monoclonal Anti-SMN1 antibody produced in mouse

clone 2F1, ascites fluid

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Synonym(s):
SMA, SMA1, SMA2, SMA3, SMN
UNSPSC Code:
12352203
NACRES:
NA.41

biological source

mouse

Quality Level

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

Gene Information

human ... SMN1(6606)

Related Categories

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

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.

WGK

WGK 3

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Regulatory Information

常规特殊物品

Certificates of Analysis (COA)

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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 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)

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