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Merck

SAB5300203

Sigma-Aldrich

Monoclonal Anti-SMN1 antibody produced in mouse

clone 2F1, ascites fluid

别名:

SMA, SMA1, SMA2, SMA3, SMN

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About This Item

分類程式碼代碼:
12352203
NACRES:
NA.41

生物源

mouse

共軛

unconjugated

抗體表格

ascites fluid

抗體產品種類

primary antibodies

無性繁殖

2F1, monoclonal

分子量

39 kDa

物種活性

monkey, human

技術

direct ELISA: 1:10,000
flow cytometry: 1:200-1:400
immunohistochemistry: 1:200-1:1,000
western blot: 1:500-1:2,000

同型

IgG1

NCBI登錄號

運輸包裝

wet ice

儲存溫度

−20°C

目標翻譯後修改

unmodified

基因資訊

human ... SMN1(6606)

一般說明

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

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).

外觀

Ascitic fluid containing 0.03% sodium azide.

免責聲明

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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儲存類別代碼

10 - Combustible liquids

水污染物質分類(WGK)

WGK 3

閃點(°F)

Not applicable

閃點(°C)

Not applicable


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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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