Affinage

LRBA

Lipopolysaccharide-responsive and beige-like anchor protein · UniProt P50851

Length
2863 aa
Mass
319.1 kDa
Annotated
2026-06-10
100 papers in source corpus 13 papers cited in narrative 13 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 6/6 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

LRBA is a BEACH domain-containing endosomal trafficking regulator that controls the surface availability of internalized membrane receptors, most prominently the immune checkpoint receptor CTLA-4 (PMID:26206937, PMID:33960403). LRBA colocalizes with CTLA-4 in endosomal vesicles and protects it from lysosomal degradation: loss of LRBA accelerates CTLA-4 turnover and lowers CTLA-4 protein in FOXP3+ regulatory and activated conventional T cells, a defect reversed by blocking lysosomal degradation with chloroquine (PMID:26206937, PMID:28159733). Mechanistically, LRBA acts upstream of Rab11 by delivering internalized CTLA-4 to Rab11+ recycling compartments for return to the cell surface; constitutively active Rab11 cannot bypass LRBA loss (PMID:33960403). This trafficking function is cell-autonomous in T cells (PMID:28611475) and underlies an immune-regulatory role, as LRBA-dependent CTLA-4 enables Tregs to suppress follicular helper T cell differentiation and limit B cell antibody production (PMID:28601686), while LRBA deficiency impairs B cell activation and autophagy (PMID:22608502). Beyond CTLA-4, LRBA negatively regulates endosomal TLR3/7/9 signaling in dendritic cells, restraining IRF3/7- and PI3K/mTORC1-dependent type I IFN production (PMID:31097594), and supports the localization of select membrane-associated proteins in sensory tissues: it promotes Golf G-protein delivery to olfactory cilia (PMID:28814779) and maintains cochlear hair cell stereocilia by sustaining radixin and Nherf2 abundance (PMID:28893864). Structurally, its tandem PH-BEACH module forms an intimate, high-affinity intramolecular unit, though the PH domain does not bind phospholipids (PMID:15554694).

Mechanistic history

Synthesis pass · year-by-year structured walk · 13 steps
  1. 1992 Low

    Established the existence of the gene as a distinct human transcript, providing the molecular entity later characterized functionally.

    Evidence Differential cDNA library screening and sequencing in teratocarcinoma cells

    PMID:1505956

    Open questions at the time
    • Identification/cloning only with no functional assay
    • No protein-level or mechanistic characterization
    • Relationship to trafficking function unestablished
  2. 2004 High

    Resolved the architecture of the signature PH-BEACH module, showing the PH and BEACH domains form a tightly associated unit but the PH domain is not a canonical phospholipid sensor.

    Evidence X-ray crystallography at 2.4 Å with SPR binding validation using FAN as a model

    PMID:15554694

    Open questions at the time
    • Function of the BEACH domain in trafficking not defined
    • No structure of full-length protein or substrate-bound state
    • Does not explain how the module engages cargo
  3. 2004 Medium

    First functional placement of LRBA in a receptor-handling pathway, linking it to cell proliferation and EGFR signaling under p53/E2F1 transcriptional control.

    Evidence Promoter reporter assays, RNAi knockdown with proliferation readout, and dominant-negative EGFR phosphorylation assay in cancer cells

    PMID:15064745

    Open questions at the time
    • EGFR pathway connection is indirect
    • Mechanism linking LRBA to EGFR phosphorylation unresolved
    • Single lab, no in vivo confirmation
  4. 2007 Medium

    Demonstrated through an ortholog that the LRBA family functions in endosomal trafficking and lysosomal delivery of cell-surface proteins, foreshadowing the human receptor-recycling role.

    Evidence C. elegans sel-2 loss-of-function genetics, fluorescence microscopy, and lipophilic dye uptake assays

    PMID:17215302

    Open questions at the time
    • Ortholog study, not direct human/mouse data
    • Notch/EGFR substrate specificity may not transfer to mammals
    • Molecular step within trafficking not defined
  5. 2012 Medium

    Established loss-of-function LRBA mutations as causative of a human immune phenotype, tying the gene to B cell activation and autophagy.

    Evidence Genetic linkage, sequencing, Western blot for protein absence, and in vitro B cell activation/autophagy assays from patients

    PMID:22608502

    Open questions at the time
    • Molecular basis of the autophagy defect not defined
    • Did not yet identify CTLA-4 as the key target
    • Single-lab patient cohort
  6. 2015 High

    Identified CTLA-4 as a key LRBA cargo and defined the mechanism: LRBA protects internalized CTLA-4 from lysosomal degradation, explaining low CTLA-4 in LRBA deficiency.

    Evidence Endosomal colocalization imaging, knockdown, chloroquine rescue, and flow cytometry across T cell subsets

    PMID:26206937

    Open questions at the time
    • Did not define which recycling compartment LRBA routes CTLA-4 through
    • Direct LRBA–CTLA-4 binding interface not mapped
    • Mechanism of cargo selection unknown
  7. 2017 High

    Showed the CTLA-4 defect is cell-autonomous in T cells and is the earliest detectable Treg abnormality, isolating the primary lesion from downstream immune dysregulation.

    Evidence CRISPR/Cas9 knockout mice and bone marrow chimeras with flow cytometry

    PMID:28611475

    Open questions at the time
    • Does not address LRBA functions outside T cells
    • Trafficking step not dissected in vivo
  8. 2017 Medium

    Connected the molecular CTLA-4 defect to immune-regulatory consequences, showing LRBA-dependent CTLA-4 enables Treg suppression of TFH differentiation and antibody production.

    Evidence In vitro TFH differentiation, Treg suppression co-culture, and B cell–TFH immunoglobulin assays

    PMID:28601686

    Open questions at the time
    • In vitro assays only
    • Single lab
    • Quantitative contribution of TFH dysregulation to disease unresolved
  9. 2017 Medium

    Provided a clinical functional test distinguishing LRBA from CTLA-4 defects, confirming the LRBA lesion lies at the trafficking/degradation step.

    Evidence Flow cytometry for surface CTLA-4 with and without lysosomal inhibitors on patient primary T cells

    PMID:28159733

    Open questions at the time
    • Single-lab patient cohort
    • Does not resolve the molecular trafficking machinery
  10. 2017 Medium

    Extended LRBA function beyond immunity, showing it maintains cochlear hair cell stereocilia by sustaining radixin and Nherf2 abundance.

    Evidence LRBA knockout mice with ABR electrophysiology, scanning electron microscopy, and immunoblot for radixin/Nherf2

    PMID:28893864

    Open questions at the time
    • Mechanism by which LRBA regulates radixin/Nherf2 levels unknown
    • Single lab
    • Trafficking versus stability mechanism not separated
  11. 2017 Medium

    Demonstrated a tissue-specific trafficking role, showing LRBA promotes Golf heterotrimeric G-protein localization to olfactory cilia and olfactory function.

    Evidence LRBA knockout mice with electro-olfactogram recordings, cilia-fraction immunoblot for Golf subunits, and behavioral assays

    PMID:28814779

    Open questions at the time
    • Mechanism of Golf delivery to cilia not defined
    • Direct interaction with Golf subunits not shown
    • Single lab
  12. 2019 High

    Established LRBA as a negative regulator of endosomal TLR signaling, broadening its endosomal role to innate immunity.

    Evidence ENU screen, Lrba knockout mice, Unc93b1 epistasis rescue, TLR stimulation, and DSS colitis model

    PMID:31097594

    Open questions at the time
    • Molecular target of LRBA within the endosomal TLR pathway unknown
    • Link to CTLA-4 trafficking mechanism unclear
  13. 2021 High

    Placed LRBA mechanistically upstream of Rab11, showing it delivers internalized CTLA-4 to Rab11+ recycling compartments rather than acting through Rab11 itself.

    Evidence Dominant-negative/constitutively active Rab mutants, LRBA knockout/knockdown in HeLa and Jurkat cells, surface CTLA-4 and colocalization assays

    PMID:33960403

    Open questions at the time
    • How LRBA hands cargo to Rab11 compartments not defined
    • Direct adaptor partners in the recycling step unidentified

Open questions

Synthesis pass · forward-looking unresolved questions
  • How the conserved PH-BEACH module recognizes and selects diverse cargoes (CTLA-4, Golf, TLR machinery, stereocilia adaptors) and what direct molecular partners mediate sorting into recycling versus lysosomal fates remain unresolved.
  • No direct LRBA-cargo binding interface mapped for any substrate
  • Adaptor/effector machinery linking LRBA to Rab11 unknown
  • Whether the multiple tissue phenotypes share one molecular mechanism unestablished

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0060090 molecular adaptor activity 2 GO:0140096 catalytic activity, acting on a protein 2
Localization
GO:0005768 endosome 3 GO:0031410 cytoplasmic vesicle 2 GO:0005929 cilium 1
Pathway
R-HSA-168256 Immune System 3 R-HSA-5653656 Vesicle-mediated transport 3 R-HSA-9609507 Protein localization 2
Partners
CTLA4GNALNHERF2RDX

Evidence

Reading pass · 13 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2015 LRBA colocalizes with CTLA4 in endosomal vesicles, and LRBA deficiency or knockdown increases CTLA4 turnover via lysosomal degradation, resulting in reduced CTLA4 protein levels in FoxP3+ regulatory T cells and activated conventional T cells. Inhibition of lysosomal degradation with chloroquine prevented CTLA4 loss in LRBA-deficient cells. Subcellular colocalization imaging, knockdown experiments, chloroquine rescue assay, flow cytometry for CTLA4 protein levels Science High 26206937
2021 LRBA is required for effective CTLA-4 recycling by delivering CTLA-4 to Rab11+ recycling compartments. In LRBA-deficient Jurkat cells, CTLA-4 recycling is markedly impaired and CTLA-4 undergoes increased lysosomal degradation. LRBA deficiency reduces CTLA-4 colocalization with Rab11, placing LRBA upstream of Rab11 in the recycling pathway. Constitutively active Rab11 could not rescue CTLA-4 recycling in LRBA-deficient cells. Dominant-negative and constitutively active Rab GTPase expression, LRBA knockdown/knockout in HeLa and Jurkat cells, surface CTLA-4 expression assay, colocalization microscopy Immunology High 33960403
2004 Crystal structure of the PH-BEACH domain of human LRBA/BGL at 2.4 Å resolution shows the PH domain has canonical PH-domain backbone fold but cannot bind phospholipids. The BEACH domain contains a core of partially extended peptide segments flanked by helices. The PH and BEACH domains associate intimately, and surface plasmon resonance (using FAN as a model) confirmed high-affinity PH-BEACH interaction (Kd ~120 nM). X-ray crystallography, surface plasmon resonance binding assay Biochemistry High 15554694
2017 LRBA deficiency in C57BL/6 mice (CRISPR/Cas9 knockout) causes a cell-autonomous reduction in CTLA-4 accumulation within CD4+ effector T cells and FOXP3+ Tregs. In young mice or chimeric mice where only half of T cells are LRBA-deficient, low CTLA-4 was the only detectable Treg abnormality. CRISPR/Cas9 gene targeting, bone marrow chimeras, flow cytometry for CTLA-4 and FOXP3 Immunology and cell biology High 28611475
2007 SEL-2, the C. elegans homolog of LRBA/neurobeachin, is a negative regulator of LIN-12/Notch activity in vulval precursor cells. Loss of sel-2 causes basolateral mislocalization and increased accumulation of LIN-12, impairs downregulation of basolateral LET-23/EGFR, and leads to aberrant FM4-64 lipophilic dye accumulation in intestinal epithelium when presented basolaterally — indicating SEL-2/LRBA is involved in endosomal trafficking and efficient delivery of cell-surface proteins to the lysosome. C. elegans genetics (loss-of-function), fluorescence microscopy, lipophilic dye uptake assay Development Medium 17215302
2019 In LRBA-deficient mice, dendritic cells exhibit excessive IRF3/7- and PI3K/mTORC1-dependent signaling and type I IFN production in response to endosomal TLR3, TLR7, and TLR9 stimulation. Knockout of Unc93b1 (required for TLR3/7/9 trafficking to endosomes) substantially reduced cytokine expression and DSS-colitis sensitivity in LRBA-deficient mice, placing LRBA as a negative regulator of endosomal TLR signaling. ENU forward genetic screen, Lrba knockout mice, Unc93b1 double-knockout epistasis, cytokine measurement by ELISA/RNA, DSS colitis model Proceedings of the National Academy of Sciences of the United States of America High 31097594
2017 LRBA deficiency leads to impaired CTLA4-mediated suppression of follicular helper T (TFH) cell differentiation. LRBA-sufficient but not LRBA-deficient regulatory T cells suppressed in vitro TFH cell differentiation in a CTLA4-dependent manner. LRBA-deficient TFH cells supported in vitro antibody production by naive B cells. In vitro TFH differentiation assay, Treg suppression co-culture assay, B cell–TFH co-culture immunoglobulin production assay The Journal of allergy and clinical immunology Medium 28601686
2017 LRBA is required for maintenance of cochlear hair cell stereociliary bundles and hearing. LRBA knockout mice show progressive sensorineural hearing loss with partial degeneration of inner and outer hair cell stereocilia during the second postnatal week. LRBA deficiency is associated with reduced abundance of radixin and Nherf2 (adaptor proteins critical for stereocilia mechanical stability) at the basal taper region of stereocilia. LRBA knockout mouse model, auditory brainstem response/electrophysiology, scanning electron microscopy of hair bundles, immunofluorescence/Western blot for radixin and Nherf2 EMBO reports Medium 28893864
2017 LRBA promotes the localization of the heterotrimeric G-protein Golf (αolf, β1, γ13 subunits) to olfactory cilia. LRBA-KO mice show markedly reduced levels (20–40% of wild-type) of all three Golf subunits in olfactory cilia, impaired olfactory electro-olfactogram responses, and smaller olfactory bulbs, without gross defects in cilia morphology or most other cilia proteins. LRBA knockout mice, electro-olfactogram recordings, immunofluorescence/Western blot for Golf subunits in cilia fractions, food-finding behavioral assay Scientific reports Medium 28814779
2004 LRBA expression is regulated by p53 (repressor) and E2F1 (activator) at the promoter level. Inhibition of LRBA by RNA interference or a dominant-negative mutant causes significant growth inhibition of cancer cells. The dominant-negative LRBA mutant affects EGFR phosphorylation, suggesting LRBA functions in the mammalian EGFR pathway. Microarray and real-time PCR for expression, promoter reporter assay (p53/E2F1), RNAi knockdown with cell proliferation assay, dominant-negative overexpression with EGFR phosphorylation assay Oncogene Medium 15064745
2017 Using lysosomal-blocking compounds (e.g., chloroquine), LRBA mutations can be functionally distinguished from direct CTLA-4 mutations: blocking lysosomal degradation rescues surface CTLA-4 in LRBA-deficient but not in CTLA-4-deficient cells, indicating the functional defect in LRBA deficiency is specifically at the trafficking/degradation step. Flow cytometry for CTLA-4 levels with and without lysosomal inhibitors, stimulation assays on primary T cells from patients Blood Medium 28159733
2012 Homozygous loss-of-function mutations in LRBA abolish LRBA protein expression and result in defective in vitro B cell activation, plasmablast formation, immunoglobulin secretion, and reduced autophagy, establishing LRBA as required for normal B cell activation and autophagy. Genetic linkage analysis, gene sequencing, Western blot (protein absence), in vitro B cell activation and proliferation assays, autophagy assays American journal of human genetics Medium 22608502
1992 CDC4L (an alias for LRBA) was identified as a novel human gene with homology to yeast CDC4, driven by an endogenous RTVL-H long terminal repeat, establishing it as a cellular gene with a ~862 bp open reading frame expressed in teratocarcinoma cells. Differential screening of cDNA library, sequencing, structural analysis of open reading frames Genomics Low 1505956

Source papers

Stage 0 corpus · 100 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2015 AUTOIMMUNE DISEASE. Patients with LRBA deficiency show CTLA4 loss and immune dysregulation responsive to abatacept therapy. Science (New York, N.Y.) 518 26206937
2012 Deleterious mutations in LRBA are associated with a syndrome of immune deficiency and autoimmunity. American journal of human genetics 390 22608502
1987 Beta-glucoside (bgl) operon of Escherichia coli K-12: nucleotide sequence, genetic organization, and possible evolutionary relationship to regulatory components of two Bacillus subtilis genes. Journal of bacteriology 239 3034860
2016 The extended phenotype of LPS-responsive beige-like anchor protein (LRBA) deficiency. The Journal of allergy and clinical immunology 211 26768763
2014 Regulatory T-cell deficiency and immune dysregulation, polyendocrinopathy, enteropathy, X-linked-like disorder caused by loss-of-function mutations in LRBA. The Journal of allergy and clinical immunology 196 25468195
2012 LPS-responsive beige-like anchor (LRBA) gene mutation in a family with inflammatory bowel disease and combined immunodeficiency. The Journal of allergy and clinical immunology 190 22721650
1990 Transcriptional antitermination in the bgl operon of E. coli is modulated by a specific RNA binding protein. Cell 168 1698125
2015 Spectrum of Phenotypes Associated with Mutations in LRBA. Journal of clinical immunology 156 26707784
1988 Regulation of the bgl operon of Escherichia coli by transcriptional antitermination. The EMBO journal 134 2846278
2019 Abatacept as a Long-Term Targeted Therapy for LRBA Deficiency. The journal of allergy and clinical immunology. In practice 128 31238161
1987 A bacterial gene involved in transcription antitermination: regulation at a rho-independent terminator in the bgl operon of E. coli. Cell 125 3301003
2019 Long-term outcome of LRBA deficiency in 76 patients after various treatment modalities as evaluated by the immune deficiency and dysregulation activity (IDDA) score. The Journal of allergy and clinical immunology 120 31887391
1987 Positive and negative regulation of the bgl operon in Escherichia coli. Journal of bacteriology 103 3294798
2017 Exaggerated follicular helper T-cell responses in patients with LRBA deficiency caused by failure of CTLA4-mediated regulation. The Journal of allergy and clinical immunology 91 28601686
1995 New beta-glucoside (bgl) genes in Bacillus subtilis: the bglP gene product has both transport and regulatory functions similar to those of BglF, its Escherichia coli homolog. Journal of bacteriology 89 7883710
2017 Identifying functional defects in patients with immune dysregulation due to LRBA and CTLA-4 mutations. Blood 87 28159733
1990 Beta-glucoside permease represses the bgl operon of Escherichia coli by phosphorylation of the antiterminator protein and also interacts with glucose-specific enzyme III, the key element in catabolite control. Proceedings of the National Academy of Sciences of the United States of America 81 2195546
2015 Autoimmune lymphoproliferative syndrome-like disease in patients with LRBA mutation. Clinical immunology (Orlando, Fla.) 79 25931386
1998 Activation of the bgl operon by adaptive mutation. Molecular biology and evolution 72 9491599
2018 Pediatric-onset Evans syndrome: Heterogeneous presentation and high frequency of monogenic disorders including LRBA and CTLA4 mutations. Clinical immunology (Orlando, Fla.) 68 29330115
2004 The histone-like nucleoid structuring protein H-NS represses the Escherichia coli bgl operon downstream of the promoter. Molecular microbiology 68 15066043
1992 Nucleotide sequences of the arb genes, which control beta-glucoside utilization in Erwinia chrysanthemi: comparison with the Escherichia coli bgl operon and evidence for a new beta-glycohydrolase family including enzymes from eubacteria, archeabacteria, and humans. Journal of bacteriology 68 1732212
1992 Strategy for detecting cellular transcripts promoted by human endogenous long terminal repeats: identification of a novel gene (CDC4L) with homology to yeast CDC4. Genomics 67 1505956
2010 BglJ-RcsB heterodimers relieve repression of the Escherichia coli bgl operon by H-NS. Journal of bacteriology 64 20952573
1997 Characterization of the negative elements involved in silencing the bgl operon of Escherichia coli: possible roles for DNA gyrase, H-NS, and CRP-cAMP in regulation. Molecular microbiology 64 9179854
2015 Atypical manifestation of LRBA deficiency with predominant IBD-like phenotype. Inflammatory bowel diseases 62 25479458
2004 Deregulated expression of LRBA facilitates cancer cell growth. Oncogene 59 15064745
1998 Lac and lambda repressors relieve silencing of the Escherichia coli bgl promoter. Activation by alteration of a repressing nucleoprotein complex. Journal of molecular biology 59 9837711
1994 Mutations in a gene encoding a new Hsp70 suppress rapid DNA inversion and bgl activation, but not proU derepression, in hns-1 mutant Escherichia coli. Journal of bacteriology 58 8300516
1998 The leuO gene product has a latent ability to relieve bgl silencing in Escherichia coli. Journal of bacteriology 57 9422614
2007 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. Development (Cambridge, England) 53 17215302
1997 BglF, the sensor of the E. coli bgl system, uses the same site to phosphorylate both a sugar and a regulatory protein. The EMBO journal 52 9303306
1996 Silencing of the Escherichia coli bgl promoter: effects of template supercoiling and cell extracts on promoter activity in vitro. Nucleic acids research 52 8710516
2017 Clinical, immunologic, molecular analyses and outcomes of iranian patients with LRBA deficiency: A longitudinal study. Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology 51 28512785
2017 Multiple Presentations of LRBA Deficiency: a Single-Center Experience. Journal of clinical immunology 50 28956255
2019 A Spectrum of Clinical Findings from ALPS to CVID: Several Novel LRBA Defects. Journal of clinical immunology 49 31432443
1988 DNA sequence of the gene scrA encoding the sucrose transport protein EnzymellScr of the phosphotransferase system from enteric bacteria: homology of the EnzymellScr and EnzymellBgl proteins. Molecular microbiology 49 28776786
2004 Crystal structure of the PH-BEACH domains of human LRBA/BGL. Biochemistry 46 15554694
1996 A mutation in a new gene, bglJ, activates the bgl operon in Escherichia coli K-12. Genetics 46 8725214
2016 LRBA deficiency with autoimmunity and early onset chronic erosive polyarthritis. Clinical immunology (Orlando, Fla.) 45 27057999
1995 Transcriptional activation of the Escherichia coli bgl operon: negative regulation by DNA structural elements near the promoter. Molecular microbiology 45 8594328
2023 Therapeutic modalities and clinical outcomes in a large cohort with LRBA deficiency and CTLA4 insufficiency. The Journal of allergy and clinical immunology 40 37595759
2021 Regulation of CTLA-4 recycling by LRBA and Rab11. Immunology 40 33960403
2001 Requirement for the molecular adapter function of StpA at the Escherichia coli bgl promoter depends upon the level of truncated H-NS protein. Molecular microbiology 37 11737635
2005 The bgl sensory system: a transmembrane signaling pathway controlling transcriptional antitermination. Current opinion in microbiology 36 15802242
1992 Experimental evidence for an alternative to directed mutation in the bgl operon. Nature 35 1557128
2016 Successful Hematopoietic Stem Cell Transplantation in a Patient with LPS-Responsive Beige-Like Anchor (LRBA) Gene Mutation. Journal of clinical immunology 34 27146671
2017 Murine LRBA deficiency causes CTLA-4 deficiency in Tregs without progression to immune dysregulation. Immunology and cell biology 32 28611475
2016 Infancy-Onset T1DM, Short Stature, and Severe Immunodysregulation in Two Siblings With a Homozygous LRBA Mutation. The Journal of clinical endocrinology and metabolism 32 26745254
2002 Post-transcriptional enhancement of Escherichia coli bgl operon silencing by limitation of BglG-mediated antitermination at low transcription rates. Molecular microbiology 31 11849549
1979 Structural studies on oncornavirus-related sequences in chicken genomic DNA: two-step analyses of EcoRI and Bgl I restriction digests and tentative mapping of a ubiquitous endogenous provirus digests and tentative mapping of a ubiquitous endogenous provirus. Proceedings of the National Academy of Sciences of the United States of America 30 221917
2018 LRBA Deficiency in a Patient With a Novel Homozygous Mutation Due to Chromosome 4 Segmental Uniparental Isodisomy. Frontiers in immunology 29 30386343
2018 2018 White Paper on Recent Issues in Bioanalysis: focus on flow cytometry, gene therapy, cut points and key clarifications on BAV (Part 3 - LBA/cell-based assays: immunogenicity, biomarkers and PK assays). Bioanalysis 29 30488726
1998 In vivo expression of the beta-glucoside (bgl) operon of Escherichia coli occurs in mouse liver. Journal of bacteriology 29 9721321
2005 Independent regulation of H-NS-mediated silencing of the bgl operon at two levels: upstream by BglJ and LeuO and downstream by DnaKJ. Microbiology (Reading, England) 28 16207917
2017 Immunological phenotype of the murine Lrba knockout. Immunology and cell biology 27 28652580
2022 2021 White Paper on Recent Issues in Bioanalysis: ISR for Biomarkers, Liquid Biopsies, Spectral Cytometry, Inhalation/Oral & Multispecific Biotherapeutics, Accuracy/LLOQ for Flow Cytometry (Part 2 - Recommendations on Biomarkers/CDx Assays Development & Validation, Cytometry Validation & Innovation, Biotherapeutics PK LBA Regulated Bioanalysis, Critical Reagents & Positive Controls Generation). Bioanalysis 26 35578974
1999 The gene bglH present in the bgl operon of Escherichia coli, responsible for uptake and fermentation of beta-glucosides encodes for a carbohydrate-specific outer membrane porin. Molecular microbiology 26 10027967
2021 2020 White Paper on Recent Issues in Bioanalysis: BAV Guidance, CLSI H62, Biotherapeutics Stability, Parallelism Testing, CyTOF and Regulatory Feedback (Part 2A - Recommendations on Biotherapeutics Stability, PK LBA Regulated Bioanalysis, Biomarkers Assays, Cytometry Validation & Innovation Part 2B - Regulatory Agencies' Inputs on Bioanalysis, Biomarkers, Immunogenicity, Gene & Cell Therapy and Vaccine). Bioanalysis 25 33511867
1999 BglG, the transcriptional antiterminator of the bgl system, interacts with the beta' subunit of the Escherichia coli RNA polymerase. Proceedings of the National Academy of Sciences of the United States of America 25 10200263
1988 DNA sequence of the gene scrA encoding the sucrose transport protein EnzymeII(Scr) of the phosphotransferase system from enteric bacteria: homology of the EnzymeII(Scr) and EnzymeII(Bgl) proteins. Molecular microbiology 25 3285123
2022 Comparing the levels of CTLA-4-dependent biological defects in patients with LRBA deficiency and CTLA-4 insufficiency. Allergy 24 35491430
2018 2018 White Paper on Recent Issues in Bioanalysis: focus on immunogenicity assays by hybrid LBA/LCMS and regulatory feedback (Part 2 - PK, PD & ADA assays by hybrid LBA/LCMS & regulatory agencies' inputs on bioanalysis, biomarkers and immunogenicity). Bioanalysis 24 30488729
2017 Multifocal gastric adenocarcinoma in a patient with LRBA deficiency. Orphanet journal of rare diseases 24 28720148
2020 IQ consortium perspective: complementary LBA and LC-MS in protein therapeutics bioanalysis and biotransformation assessment. Bioanalysis 23 32096432
2020 Clinical Phenotypes and Immunological Characteristics of 18 Egyptian LRBA Deficiency Patients. Journal of clinical immunology 22 32506362
2009 Fate of the H-NS-repressed bgl operon in evolution of Escherichia coli. PLoS genetics 22 19266030
2022 The ABACHAI clinical trial protocol: Safety and efficacy of abatacept (s.c.) in patients with CTLA-4 insufficiency or LRBA deficiency: A non controlled phase 2 clinical trial. Contemporary clinical trials communications 21 36262801
2019 Chaetomella raphigera β-glucosidase D2-BGL has intriguing structural features and a high substrate affinity that renders it an efficient cellulase supplement for lignocellulosic biomass hydrolysis. Biotechnology for biofuels 21 31700541
2009 An evolutionarily conserved nested gene pair - Mab21 and Lrba/Nbea in metazoan. Genomics 21 19482073
2006 Differential dependence of StpA on H-NS in autoregulation of stpA and in regulation of bgl. Journal of bacteriology 21 16980475
2019 Enhanced susceptibility to chemically induced colitis caused by excessive endosomal TLR signaling in LRBA-deficient mice. Proceedings of the National Academy of Sciences of the United States of America 20 31097594
2018 Rapid Flow Cytometry-Based Test for the Diagnosis of Lipopolysaccharide Responsive Beige-Like Anchor (LRBA) Deficiency. Frontiers in immunology 20 29740429
2017 The BEACH protein LRBA is required for hair bundle maintenance in cochlear hair cells and for hearing. EMBO reports 20 28893864
2009 Modulation of transcription antitermination in the bgl operon of Escherichia coli by the PTS. Proceedings of the National Academy of Sciences of the United States of America 20 19633194
2000 Mechanism of catabolite repression in the bgl operon of Escherichia coli: involvement of the anti-terminator BglG, CRP-cAMP and EIIAGlc in mediating glucose effect downstream of transcription initiation. Genes to cells : devoted to molecular & cellular mechanisms 20 10792463
1999 BglF, the Escherichia coli beta-glucoside permease and sensor of the bgl system: domain requirements of the different catalytic activities. Journal of bacteriology 20 9882659
1979 Sequence-specific endonuclease Bgl I. Modification of lysine and arginine residues of the homogeneous enzyme. The Journal of biological chemistry 20 457653
2021 Improvements of the productivity and saccharification efficiency of the cellulolytic β-glucosidase D2-BGL in Pichia pastoris via directed evolution. Biotechnology for biofuels 19 34059121
2004 Analysis of bgl operon structure and characterization of beta-glucosidase from Pectobacterium carotovorum subsp. carotovorum LY34. Bioscience, biotechnology, and biochemistry 19 15564664
2001 Mu and IS1 transpositions exhibit strong orientation bias at the Escherichia coli bgl locus. Journal of bacteriology 19 11344140
2000 Antagonistic control of the Escherichia coli bgl promoter by FIS and CAP in vitro. Molecular microbiology 19 10760165
2021 Different Apples, Same Tree: Visualizing Current Biological and Clinical Insights into CTLA-4 Insufficiency and LRBA and DEF6 Deficiencies. Frontiers in pediatrics 18 33996698
2017 Sodium Lactate Negatively Regulates Shewanella putrefaciens CN32 Biofilm Formation via a Three-Component Regulatory System (LrbS-LrbA-LrbR). Applied and environmental microbiology 18 28500045
2011 The β-glucoside (bgl) operon of Escherichia coli is involved in the regulation of oppA, encoding an oligopeptide transporter. Journal of bacteriology 18 22020646
1999 rpoS function is essential for bgl silencing caused by C-terminally truncated H-NS in Escherichia coli. Journal of bacteriology 18 10515915
1998 BglF, the sensor of the bgl system and the beta-glucosides permease of Escherichia coli: evidence for dimerization and intersubunit phosphotransfer. Biochemistry 18 9628733
2022 Leptospira spp. strains associated with Bovine Genital Leptospirosis (BGL). Microbial pathogenesis 17 36309182
2018 The imbalance of circulating T helper subsets and regulatory T cells in patients with LRBA deficiency: Correlation with disease severity. Journal of cellular physiology 17 29806698
2018 Polyautoimmunity in Patients with LPS-Responsive Beige-Like Anchor (LRBA) Deficiency. Immunological investigations 16 29528757
2003 The LicT protein acts as both a positive and a negative regulator of loci within the bgl regulon of Streptococcus mutans. Microbiology (Reading, England) 16 12724394
2023 2022 White Paper on Recent Issues in Bioanalysis: Enzyme Assay Validation, BAV for Primary End Points, Vaccine Functional Assays, Cytometry in Tissue, LBA in Rare Matrices, Complex NAb Assays, Spectral Cytometry, Endogenous Analytes, Extracellular Vesicles Part 2 - Recommendations on Biomarkers/CDx, Flow Cytometry, Ligand-Binding Assays Development & Validation; Emerging Technologies; Critical Reagents Deep Characterization. Bioanalysis 15 37584363
2019 Combined Cell Surface Display of β-d-Glucosidase (BGL), Maltose Transporter (MAL11), and Overexpression of Cytosolic Xylose Reductase (XR) in Saccharomyces cerevisiae Enhance Cellobiose/Xylose Coutilization for Xylitol Bioproduction from Lignocellulosic Biomass. Biotechnology journal 15 31283105
2017 Agammaglobulinaemia despite terminal B-cell differentiation in a patient with a novel LRBA mutation. Clinical & translational immunology 15 28690850
2017 The BEACH Protein LRBA Promotes the Localization of the Heterotrimeric G-protein Golf to Olfactory Cilia. Scientific reports 15 28814779
2004 The protease Lon and the RNA-binding protein Hfq reduce silencing of the Escherichia coli bgl operon by H-NS. Journal of bacteriology 15 15090512
2021 Pulmonary manifestations of immune dysregulation in CTLA-4 haploinsufficiency and LRBA deficiency. Pediatric pulmonology 14 33710794
2018 Respiratory manifestations in LPS-responsive beige-like anchor (LRBA) protein-deficient patients. European journal of pediatrics 14 29777306
2018 LRBA in the endomembrane system. Colombia medica (Cali, Colombia) 14 30410199
2002 Silencing of the Escherichia coli bgl operon by RpoS requires Crl. Microbiology (Reading, England) 14 12177351

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