Reactivity: | Human, Mouse |
Applications: | WB, ELISA |
Host Species: | Rabbit |
Clonality: | Polyclonal antibody |
Gene Name: | selenoprotein N |
Gene Symbol: | SELENON |
Synonyms: | RSS; CFTD; SELN; CMYP3; MDRS1; RSMD1; SEPN1 |
Gene ID: | 57190 |
UniProt ID: | Q9NZV5 |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 341-590 of human SEPN1 (NP_065184.2). |
Dilution: | WB 1:500-1:2000 |
Purification Method: | Affinity purification |
Concentration: | 2.87 mg/ml |
Buffer: | Buffer: PBS with 0.01% thimerosal,50% glycerol,pH7.3. |
Storage: | Store at -20°C. Avoid freeze/thaw cycles. |
Documents: | Manual-SELENON antibody |
Background
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.
Images
![]() | Western blot analysis of various lysates using SEPN1 Rabbit pAb (A15012) at 1:1000 dilution. Secondary antibody: HRP-conjugated Goat anti-Rabbit IgG (H+L) (AS014) at 1:10000 dilution. Lysates/proteins: 25μg per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 5S. |
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