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Merck
CN

AV47656

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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关于此项目

NACRES:
NA.43
UNSPSC Code:
12352203
Conjugate:
unconjugated
Clone:
polyclonal
Application:
WB
Citations:
2
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biological source

rabbit

conjugate

unconjugated

antibody form

affinity isolated antibody

antibody product type

primary antibodies

clone

polyclonal

form

buffered aqueous solution

mol wt

58 kDa

species reactivity

horse, bovine, human

concentration

0.5 mg - 1 mg/mL

technique(s)

western blot: suitable

NCBI accession no.

UniProt accession no.

shipped in

wet ice

storage temp.

−20°C

target post-translational modification

unmodified

Quality Level

Gene Information

human ... SEPN1(57190)

General description

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.

Immunogen

Synthetic peptide directed towards the C terminal region of human SEPN1

Application

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

Biochem/physiol Actions

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.

Physical form

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

Other Notes

Synthetic peptide located within the following region: NYFLDITSVKPEEIESNLFSFSSTFEDPSTATYMQFLKEGLRRGLPLLQP

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

常规特殊物品
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分析证书(COA)

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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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