APECED polyclonal, anti-human

APECED polyclonal, anti-human

€347.00
In stock
SKU
252014
Catalog Nr.: 252014
Size: 0.1 mg
Isotype: Rabbit Ig
Applications: E, WB
Protein Family: Ubiquitins

Pathway and Disease: Folding, Sorting and Degradation, Immune System

Description:
Autoimmune regulator (APECED) is a transcriptional regulator that binds to DNA as a dimer or as a tetramer, but not as a monomer. APECED binds to G-doublets in an A/T-rich environment; the preferred motif is a tandem repeat of 5'-. ATTGGTTA-3' combined with a 5'-TTATTA-3' box. APECED binds to chromatin and interacts selectively with histone H3 that is not methylated at 'Lys-4', not phosphorylated at 'Thr-3' and not methylated at 'Arg-2'. APECED functions as a sensor of histone H3 modifications that are important for the epigenetic regulation of gene expression. APECED functions as a transcriptional activator and promotes the expression of otherwise tissue-specific self-antigens in the thymus, which is important for self tolerance and the avoidance of autoimmune reactions. Defects in APECED are a cause of autoimmune poly-endocrinopathy candidiasis ectodermal dystrophy (APECED), also known as autoimmune polyglandular syndrome type I (APS-1). APECED is an autosomal recessive disease characterized by: (1) autoimmune polyendocrinopathies; (2) chronic mucocutaneous candidiasis; and (3) ectodermal dystrophies. In addition, a high proportion of patients develop squamous cell carcinoma of the oral mucosa. The disease is reported worldwide but is exceptionally prevalent among the Finnish population (incidence 1:25000) and the Iranian jews (incidence 1:9000).

Alternate Names: Autoimmune regulator, Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy protein, APECED protein, AIRE, APECED

Application Notes: E: 1:10,000-1:50,000; WB: 1:100-1:500

Accession No.: O43918

Antigen: KLH-conjugated synthetic peptide encompassing a sequence within the C-term region of human APECED.

Format: Each vial contains 0.1 mg IgG in 0.1 ml (1 mg/ml) of PBS pH7.4 with 0.09% sodium azide. Antibody was purified by Protein-G affinity chromatography.

Storage:
Store at -20°C. Minimize freeze-thaw cycles. Product is guaranteed one year from the date of shipment.
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