Anti-SELENON Antibody Picoband®

SKU: A31971-1

Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human

Applications: WB,Flow Cytometry,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: SELENON

Specificity:

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.

Immunogen: E.coli-derived human SELENON recombinant protein (Position: H260-P590).

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Bouman, K., Gubbels, M., van den Heuvel, F. M. A., Groothuis, J. T., Erasmus, C. E., Nijveldt, R., Udink Ten Cate, F. E. A., Voermans, N. C. Cardiac involvement in two rare neuromuscular diseases: LAMA2-related muscular dystrophy and SELENON-related myopathy. Neuromusc. Disord. 32: 635-642, 2022. 2. Castets, P., Bertrand, A. T., Beuvin, M., Ferry, A., Le Grand, F., Castets, M., Chazot, G., Rederstorff, M., Krol, A., Lescure, A., Romero, N. B., Guicheney, P., Allamand, V. Satellite cell loss and impaired muscle regeneration in selenoprotein N deficiency. Hum. Molec. Genet. 20: 694-704, 2011. 3. Clarke, N. F., Kidson, W., Quijano-Roy, S., Estournet, B., Ferreiro, A., Guicheney, P., Manson, J. I., Kornberg, A. J., Shield, L. K., North, K. N. SEPN1: associated with congenital fiber-type disproportion and insulin resistance. Ann. Neurol. 59: 546-552, 2006.

Uniprot ID: Q9NZV5

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 66-70 kDa

Calculated Molecular Weight: 14377 MW

Gene ID: 57190

Protein Name: Selenoprotein N

Gene Full Name: selenoprotein N

Synonyms: MDRS1; RSMD1; RSS; SELENON; Selenoprotein N; selenoprotein N, 1; SELN; SEPN1