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Gene Review

LRBA  -  LPS-responsive vesicle trafficking, beach...

Homo sapiens

Synonyms: BGL, Beige-like protein, CDC4-like protein, CDC4L, CVID8, ...
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Disease relevance of LRBA


    Deleterious Mutations in LRBA are Associated with a Syndrome of Immune Deficiency and Autoimmunity in human


High impact information on LRBA

  • Our observations indicate that SEL-2/neurobeachin/LRBA is involved in endosomal traffic and may be involved in efficient delivery of cell surface proteins to the lysosome [1].
  • We hypothesize that LBA/CHS1/BG proteins function in polarized vesicle trafficking by guiding intracellular vesicles to activated receptor complexes and thus facilitate polarized secretion and/or membrane deposition of immune effector molecules [2].
  • Deregulated expression of LRBA facilitates cancer cell growth [3].
  • LRBA expression is induced by mitogens in lymphoid and myeloid cells [3].
  • Furthermore, inhibition of LRBA expression by RNA interference, or inhibition of its function by a dominant-negative mutant, leads to significant growth inhibition of cancer cells, demonstrating that deregulated expression of LRBA contributes to the altered growth properties of a cancer cell [3].

Chemical compound and disease context of LRBA

  • In addition, BGL could agglutinate a range of micro-organisms and yeast cells, with the exception of some fish pathogens, such as Aeromonas hydrophila (strains: PPD 134/91 and PPD 11/90) and Vibrio harveyi (strain: W618) [4].

Biological context of LRBA


Anatomical context of LRBA

  • For specific interaction with a hepatic carcinoma cell line (HepG2), CM-curdlan was additionally conjugated with lactobionic acid (LBA; galactose moiety) (5.5 LBA molecules per hundred glucose units) [9].
  • Five lectins (LAA, STA, DBA, GSA-II, and LBA) reacted with nuclei of the cytotrophoblast [10].
  • Localisation of BGL by fluorescein isothiocyanate (FITC)-labelled antibodies revealed that the lectin is associated with the cell surface of fish leukocytes [4].
  • Haemagglutination assay showed that BGL is more specific for rabbit than mouse, chicken, rat or guinea pig erythrocytes, and haemagglutination was Ca2+-dependent [4].
  • Flow cytometric analyses showed that all but three (DBA, LBA and LPA) partially or totally stained lymphocyte surfaces [11].

Associations of LRBA with chemical compounds

  • In contrast, 4c, which is prepared from a 1:2 molar mixture of B(OH)(3) and tetrachlorocatechol, is effective as a Lewis acid-assisted Brønsted acid (LBA) catalyst for Ritter reaction [12].
  • HepG2 was strongly luminated by ligand-receptor interactions with fluorescence-labeled LBA/CM-curdlan/SU hydrogel nanoparticles [9].
  • We measured ER and PgR by both ligand binding (LBA) and immunohistochemical (IHC) assays in 34 tumors from patients on tamoxifen, 30 of whom were displaying resistance to the drug [13].
  • In the present study, to obtain an insight into the contributions of 2-HH to protein modifications during lipid peroxidation, a lysine-containing dipeptide, N(alpha)-hippuryllysine (N-benzoylglycyl-L-lysine, BGL), was reacted with 2-HH at neutral pH [14].
  • The initial complex lacks coordination of the LBA carboxylate (C-1) and is bound by the "2,3,5" hydroxyl groups [15].

Other interactions of LRBA


Analytical, diagnostic and therapeutic context of LRBA

  • Here, we show by microarray and real-time PCR analyses that LRBA is overexpressed in several different cancers relative to their normal tissue controls [3].
  • Electrophoretic analyses and mass spectrometric study of purified BGL showed that the lectin is composed of two isoforms with native molecular masses estimated to be 65 and 66 kDa, and two subunits of 32 and 34 kDa on SDS-PAGE under non-reducing conditions [4].
  • Lactobionic acid, [4-beta-(galactosido)-D-gluconic acid] = LBA, is the major component of the Wisconsin organ transplantation preservant fluid and may suppress oxygen radical-induced tissue damage upon reperfusion by the control of FeII autoxidation [15].
  • Titration data shows the removal of three protons from LBA through pH 5 and an additional proton from pH 6 to 9 which is indicative of the [FeIII(LBA)(OH)(H2O)]- formulation with LBA donating at the "1,2,3,6" positions [15].
  • Laser balloon angioplasty: a review of the technique and clinical applications. LBA Study Group [18].



  1. SEL-2, the C. elegans neurobeachin/LRBA homolog, is a negative regulator of lin-12/Notch activity and affects endosomal traffic in polarized epithelial cells. de Souza, N., Vallier, L.G., Fares, H., Greenwald, I. Development (2007) [Pubmed]
  2. Identification of a novel lipopolysaccharide-inducible gene with key features of both A kinase anchor proteins and chs1/beige proteins. Wang, J.W., Howson, J., Haller, E., Kerr, W.G. J. Immunol. (2001) [Pubmed]
  3. Deregulated expression of LRBA facilitates cancer cell growth. Wang, J.W., Gamsby, J.J., Highfill, S.L., Mora, L.B., Bloom, G.C., Yeatman, T.J., Pan, T.C., Ramne, A.L., Chodosh, L.A., Cress, W.D., Chen, J., Kerr, W.G. Oncogene (2004) [Pubmed]
  4. Isolation and characterisation of a serum lectin from blue gourami, Trichogaster trichopterus (Pallus). Fock, W.L., Chen, C.L., Lam, T.J., Sin, Y.M. Fish Shellfish Immunol. (2000) [Pubmed]
  5. Strategy for detecting cellular transcripts promoted by human endogenous long terminal repeats: identification of a novel gene (CDC4L) with homology to yeast CDC4. Feuchter, A.E., Freeman, J.D., Mager, D.L. Genomics (1992) [Pubmed]
  6. Enantio- and diastereoselective stepwise cyclization of polyprenoids induced by chiral and achiral LBAs. A new entry to (-)-ambrox, (+)-podocarpa-8,11,13-triene diterpenoids, and (-)-tetracyclic polyprenoid of sedimentary origin. Ishihara, K., Ishibashi, H., Yamamoto, H. J. Am. Chem. Soc. (2002) [Pubmed]
  7. Liquid-phase binding assay of human chorionic gonadotropin using high-performance liquid chromatography. Nakamura, K., Satomura, S., Matsuura, S. Anal. Chem. (1993) [Pubmed]
  8. A new approach in the evaluation of chromosome variants in man. II. Pairs without Q or C (qh) variants. Azumi, J., Nakagome, Y., Oka, S., Matsunaga, E. Hum. Genet. (1980) [Pubmed]
  9. Self-assembled hydrogel nanoparticles from curdlan derivatives: characterization, anti-cancer drug release and interaction with a hepatoma cell line (HepG2). Na, K., Park, K.H., Kim, S.W., Bae, Y.H. Journal of controlled release : official journal of the Controlled Release Society. (2000) [Pubmed]
  10. Lectin histochemistry of human placenta. Lee, M.C., Damjanov, I. Differentiation (1984) [Pubmed]
  11. Distribution of lectin receptors in the human hyperplastic tonsil: histochemical and flow cytometric analyses. Sarker, A.B., Akagi, T., Yoshino, T., Fujiwara, K., Nose, S. Acta Med. Okayama (1993) [Pubmed]
  12. 4,5,6,7-Tetrachlorobenzo[d][1,3,2]dioxaborol- 2-ol as an Effective Catalyst for the Amide Condensation of Sterically Demanding Carboxylic Acids. Maki, T., Ishihara, K., Yamamoto, H. Org. Lett. (2006) [Pubmed]
  13. Measurement of steroid hormone receptors in breast cancer patients on tamoxifen. Encarnación, C.A., Ciocca, D.R., McGuire, W.L., Clark, G.M., Fuqua, S.A., Osborne, C.K. Breast Cancer Res. Treat. (1993) [Pubmed]
  14. Reaction of N(alpha)-hippuryllysine with 2-hydroxyheptanal: a model for lysine-directed protein modifications by lipid peroxidation. Itakura, K., Uchida, K. Chem. Phys. Lipids (2003) [Pubmed]
  15. Lactobionic and gluconic acid complexes of FeII and FeIII; control of oxidation pathways by an organ transplantation preservant. Shepherd, R.E., Isaacson, Y., Chensny, L., Zhang, S., Kortes, R., John, K. J. Inorg. Biochem. (1993) [Pubmed]
  16. BCL8 is a novel, evolutionarily conserved human gene family encoding proteins with presumptive protein kinase A anchoring function. Dyomin, V.G., Chaganti, S.R., Dyomina, K., Palanisamy, N., Murty, V.V., Dalla-Favera, R., Chaganti, R.S. Genomics (2002) [Pubmed]
  17. Prognostic value of pS2 protein and receptors for epidermal growth factor (EGF-R), insulin-like growth factor-1 (IGF-1-R) and somatostatin (SS-R) in patients with breast and ovarian cancer. Foekens, J.A., van Putten, W.L., Portengen, H., Rodenburg, C.J., Reubi, J.C., Berns, P.M., Henzen-Logmans, S.C., van der Burg, M.E., Alexieva-Figusch, J., Klijn, J.G. J. Steroid Biochem. Mol. Biol. (1990) [Pubmed]
  18. Laser balloon angioplasty: a review of the technique and clinical applications. LBA Study Group. Jenkins, R.D., Spears, J.R. The Journal of invasive cardiology. (1990) [Pubmed]
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