LRBA Antibody

LRBA (lipopolysaccharide responsive beige-like anchor protein) is a 300 kDa intracellular protein that is a member of the PH-BEACH-WD40 protein family (1-3). Its BEACH domain contains an A-kinase anchoring protein (AKAP) motif, known to have an affinity for signaling enzymes (1,3). LRBA has been found to be overexpressed in several different cancers, such as renal, pancreas, colorectal, and lung (2). LRBA deficiency, caused by various mutations, can cause common variable immune deficiency (CVID) as well as other immune dysregulations (3,5). LRBA plays an immunoregulatory role to CTLA-4, protecting it from being degraded within lysosomes (4,6). In LRBA deficiency, CTLA-4 is synthesized normally, but is not delivered to Rab GTPase recycling compartments, instead resulting in enhanced lysosome degradation (4,6,7). 1.Wang, J.W. et al. (2001) J Immunol 166, 4586-95. 2.Wang, J.W. et al. (2004) Oncogene 23, 4089-97. 3.Lopez-Herrera, G. et al. (2012) Am J Hum Genet 90, 986-1001. 4.Lo, B. et al. (2015) Science 349, 436-40. 5.Gámez-Díaz, L. et al. (2016) J Allergy Clin Immunol 137, 223-230. 6.Hou, T.Z. et al. (2017) Blood 129, 1458-1468. 7.Janman, D. et al. (2021) Immunology 164, 106-119.