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Merck

AV47656

Sigma-Aldrich

Anti-SEPN1 antibody produced in rabbit

affinity isolated antibody

Synonym(e):

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

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About This Item

UNSPSC-Code:
12352203
NACRES:
NA.43

Biologische Quelle

rabbit

Qualitätsniveau

Konjugat

unconjugated

Antikörperform

affinity isolated antibody

Antikörper-Produkttyp

primary antibodies

Klon

polyclonal

Form

buffered aqueous solution

Mol-Gew.

58 kDa

Speziesreaktivität

horse, bovine, human

Konzentration

0.5 mg - 1 mg/mL

Methode(n)

western blot: suitable

NCBI-Hinterlegungsnummer

UniProt-Hinterlegungsnummer

Versandbedingung

wet ice

Lagertemp.

−20°C

Posttranslationale Modifikation Target

unmodified

Angaben zum Gen

human ... SEPN1(57190)

Allgemeine Beschreibung

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

Anwendung

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

Biochem./physiol. Wirkung

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.

Sequenz

Synthetic peptide located within the following region: NYFLDITSVKPEEIESNLFSFSSTFEDPSTATYMQFLKEGLRRGLPLLQP

Physikalische Form

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

Haftungsausschluss

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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Lagerklassenschlüssel

10 - Combustible liquids

WGK

WGK 3

Flammpunkt (°F)

Not applicable

Flammpunkt (°C)

Not applicable


Analysenzertifikate (COA)

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Die Dokumentenbibliothek aufrufen

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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