The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
应用说明ELISA: Use at a concentration of 0.5 - 1.0 µg/ml. Detection limit of ~0.12 ng/well of recombinant human IL12 Rbeta1.
WB: Use at a concentration of 0.1 - 0.2 µg/ml. Detects human IL12Rbeta1 at 20 ng/lane under non-reducing and reducing conditions. Predicted molecular weight: 73 kDa.
Not tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
功能Functions as an interleukin receptor which binds interleukin-12 with low affinity and is involved in IL12 transduction. Associated with IL12RB2 it forms a functional, high affinity receptor for IL12. Associates also with IL23R to form the interleukin-23 receptor which functions in IL23 signal transduction probably through activation of the Jak-Stat signaling cascade.
疾病相关Defects in IL12RB1 are a cause of mendelian susceptibility to mycobacterial disease (MSMD) [MIM:209950]; also known as familial disseminated atypical mycobacterial infection. This rare condition confers predisposition to illness caused by moderately virulent mycobacterial species, such as Bacillus Calmette-Guerin (BCG) vaccine and environmental non-tuberculous mycobacteria, and by the more virulent Mycobacterium tuberculosis. Other microorganisms rarely cause severe clinical disease in individuals with susceptibility to mycobacterial infections, with the exception of Salmonella which infects less than 50% of these individuals. The pathogenic mechanism underlying MSMD is the impairment of interferon-gamma mediated immunity, whose severity determines the clinical outcome. Some patients die of overwhelming mycobacterial disease with lepromatous-like lesions in early childhood, whereas others develop, later in life, disseminated but curable infections with tuberculoid granulomas. MSMD is a genetically heterogeneous disease with autosomal recessive, autosomal dominant or X-linked inheritance.
序列相似性Belongs to the type I cytokine receptor family. Type 2 subfamily. Contains 5 fibronectin type-III domains.
结构域The WSXWS motif appears to be necessary for proper protein folding and thereby efficient intracellular transport and cell-surface receptor binding. The box 1 motif is required for JAK interaction and/or activation.