Cat. No.: 524 003
Amount: 50 µg
Price:
$380.00
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| Cat. No. 524 003 |
50 µg specific antibody, lyophilized. Affinity purified with the immunogen. Albumin and azide were added for stabilization. For reconstitution add 50 µl H2O to get a 1mg/ml solution in PBS. Then aliquot and store at -20°C to -80°C until use. Antibodies should be stored at +4°C when still lyophilized. Do not freeze! |
| Applications | |
| Immunogen | Recombinant protein corresponding to residues near the central region of mouse LRBA (UniProt Id: Q9ESE1) |
| Reactivity |
Reacts with: mouse (Q9ESE1). Other species not tested yet. |
| Specificity | K.O. validated PubMed: 28814779 |
| Remarks |
WB: Due to its large size, LRBA requires special gel-electrophoresis and Western blot protocols for visualization by immunoblotting. Excellent results can be obtained with NuPAGE 3-8% TRIS-Acetate gradient gels from Invitrogen. |
| Data sheet | 524_003.pdf |
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LRBA (Lipopolysaccharide-Responsive and Beige-Like Anchor Protein) is a member of the BEACH-protein family. It is a large cytosolic protein comprising several functional domains, including the Plekstrin Homology (PH)-like domain, BEACH domain, and multiple WD40 repeats (1).
LRBA is essential for the proper functioning of immune cells, particularly B cells and T cells. It has been shown to regulate the recycling of the immune receptor CTLA-4, which is crucial for T cell activation and downregulation (2, 3). LRBA deficiency manifests as immunodeficiency, autoimmune diseases, and various hematological disorders, like reduced levels of B cells, impaired immunoglobulin production and more (4).
Dysfunctional LRBA is also connected to several CNS disorders like sensorineural hearing loss (5), cerebral granulomatous lesions, nerve demyelination and atrophy (6).
LRBA is widely expressed in multiple tissues including lymphoid organs, the gastrointestinal tract, kidney, the pancreas, and neuronal cells (7).