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Merck
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主要文件

AV47656

Sigma-Aldrich

Anti-SEPN1 antibody produced in rabbit

affinity isolated antibody

别名:

Anti-FLJ24021, Anti-MDRS1, Anti-RSMD1, Anti-RSS, Anti-SELN, Anti-Selenoprotein N,1

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About This Item

分類程式碼代碼:
12352203
NACRES:
NA.43

生物源

rabbit

品質等級

共軛

unconjugated

抗體表格

affinity isolated antibody

抗體產品種類

primary antibodies

無性繁殖

polyclonal

形狀

buffered aqueous solution

分子量

58 kDa

物種活性

horse, bovine, human

濃度

0.5 mg - 1 mg/mL

技術

western blot: suitable

NCBI登錄號

UniProt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 ... SEPN1(57190)

一般說明

SEPN1 codes for selenoprotein N that is involved in redox homeostasis. It protects cells against oxidative stress. Mutations in this gene have been linked to SEPN1-related myopathy, multiminicore disease, and congenital muscular dystrophy.
Rabbit anti-SEPN1 antibody recognizes human, mouse, pig, and bovine SEPN1.

免疫原

Synthetic peptide directed towards the C terminal region of human SEPN1

應用

Rabbit anti-SEPN1 antibody is suitable for western blot applications at a concentration of 1μg/ml.

生化/生理作用

SEPN1 is a selenoprotein, which contains a selenocysteine (Sec) residue at its active site. Mutations in SEPN1 gene cause the classical phenotype of multiminicore disease and congenital muscular dystrophy with spinal rigidity and restrictive respiratory syndrome.This gene encodes a selenoprotein, which contains a selenocysteine (Sec) residue at its active site. The selenocysteine is encoded by the UGA codon that normally signals translation termination. The 3′ UTR of selenoprotein genes have a common stem-loop structure, the sec insertion sequence (SECIS), that is necessary for the recognition of UGA as a Sec codon rather than as a stop signal. Mutations in this gene cause the classical phenotype of multiminicore disease and congenital muscular dystrophy with spinal rigidity and restrictive respiratory syndrome. Two alternatively spliced transcript variants encoding distinct isoforms have been found for this gene.

序列

Synthetic peptide located within the following region: NYFLDITSVKPEEIESNLFSFSSTFEDPSTATYMQFLKEGLRRGLPLLQP

外觀

Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

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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Sandrine Arbogast et al.
Annals of neurology, 65(6), 677-686 (2009-06-27)
Mutations of the selenoprotein N gene (SEPN1) cause SEPN1-related myopathy (SEPN1-RM), a novel early-onset muscle disorder formerly divided into four different nosological categories. Selenoprotein N (SelN) is the only selenoprotein involved in a genetic disease; its function being unknown, no
M Scoto et al.
Neurology, 76(24), 2073-2078 (2011-06-15)
To assess the clinical course and genotype-phenotype correlations in patients with selenoprotein-related myopathy (SEPN1-RM) due to selenoprotein N1 gene (SEPN1) mutations for a retrospective cross-sectional study. Forty-one patients aged 1-60 years were included. Clinical data including scoliosis, respiratory function, and

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