SELENON Antilichaam - C-terminaal gebied: Biotine (ARP47655 P050-Biotine)

Selenoprotein N

RSS, CFTD, SELN, MDRS1, RSMD1, SEPN1

57190

Q9NZV5

NP_065184

Human, Mouse, Rat, Cow, Guinea Pig, Horse, Zebrafish

The immunogen is a synthetic peptide directed towards the C terminal region of human SEPN1

This gene encodes a glycoprotein that is localized in the endoplasmic reticulum. It plays an important role in cell protection against oxidative stress, and in the regulation of redox-related calcium homeostasis. Mutations in this gene are associated with early onset muscle disorders, referred to as SEPN1-related myopathy. SEPN1-related myopathy consists of 4 autosomal recessive disorders, originally thought to be separate entities: rigid spine muscular dystrophy (RSMD1), the classical form of multiminicore disease, desmin related myopathy with Mallory-body like inclusions, and congenital fiber-type disproportion (CFTD) . This protein is a selenoprotein, containing the rare amino acid selenocysteine (Sec) . Sec is encoded by the UGA codon, which normally signals translation termination. The 3' UTRs of selenoprotein mRNAs contain a conserved stem-loop structure, designated the Sec insertion sequence (SECIS) element, that is necessary for the recognition of UGA as a Sec codon, rather than as a stop signal. A second stop-codon redefinition element (SRE) adjacent to the UGA codon has been identified in this gene (PMID:15791204) . SRE is a phylogenetically conserved stem-loop structure that stimulates readthrough at the UGA codon, and augments the Sec insertion efficiency by SECIS. Alternatively spliced transcript variants have been found for this gene.

ELAVL1; UBC

Biotin

Polyclonal Antibody

WB, IHC

Affinity Purified

0.5 mg/ml

Cow: 100%; Guinea Pig: 100%; Horse: 100%; Human: 100%; Mouse: 93%; Rat: 100%; Zebrafish: 79%

Liquid. Purified antibody supplied in 1x PBS buffer.

All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.

62kDa

Okamoto, Y., (2006) Neurogenetics 7 (3), 175-183

Wet Ice

590

SELENON

SELENON

Rabbit

Selenoprotein N

NM_020451

ANYFLDITSVKPEEIESNLFSFSSTFEDPSTATYMQFLKEGLRRGLPLLQ