Killing three birds with one BPI: Bactericidal, opsonic, and anti-inflammatory functions

Aichele, 2006, Expression and antimicrobial function of bactericidal permeability- increasing protein in cystic fibrosis patients, Infect. Immun., 74, 10.1128/IAI.02066-05 Kerem, 1990, Pulmonary function and clinical course in patients with cystic fibrosis after pulmonary colonization with Pseudomonas aeruginosa, J. Pediatr., 116, 10.1016/S0022-3476(05)82653-8 Pamukcu, 1995, Effects of Pseudomonas aeruginosa colonization on lung function and anthropometric variables in children with cystic fibrosis, Pediatr. Pulmonol., 19, 10.1002/ppul.1950190103 Kosorok, 2001, Acceleration of lung disease in children with cystic fibrosis after Pseudomonas aeruginosa acquisition, Pediatr. Pulmonol., 32, 10.1002/ppul.2009.abs Henry, 1992, Mucoid Pseudomonas aeruginosa is a marker of poor survival in cystic fibrosis, Pediatr. Pulmonol., 12, 10.1002/ppul.1950120306 Skopelja-Gardner, 2019, Autoimmunity to bactericidal/permeability-increasing protein in bronchiectasis exhibits a requirement for Pseudomonas aeruginosa IgG response, Eur. Respir. J., 53, 10.1183/13993003.01891-2018 Tian, 2018, BPI-ANCA in chronic obstructive pulmonary disease with pulmonary Pseudomonas aeruginosa colonisation: a novel indicator of poor prognosis, Br. J. Biomed. Sci., 75, 10.1080/09674845.2018.1512260 Schultz, 2007, A novel role for the bactericidal/permeability increasing protein in interactions of gram-negative bacterial outer membrane blebs with dendritic cells, J. Immunol., 179, 10.4049/jimmunol.179.4.2477 Jennette, 2014, Pathogenesis of antineutrophil cytoplasmic autoantibody-mediated disease, Nat. Rev. Rheumatol., 10, 10.1038/nrrheum.2014.103 Theprungsirikul, 2020, Dissociation of systemic and mucosal autoimmunity in cystic fibrosis, J. Cyst. Fibros., 19, 196, 10.1016/j.jcf.2019.06.006 Weiss, 1987, Cellular and subcellular localization of the bactericidal/permeability-increasing protein of neutrophils, Blood, 69, 10.1182/blood.V69.2.652.652 Weiss, 1978, Purification and characterization of a potent bactericidal and membrane active protein from the granules of human polymorphonuclear leukocytes, J. Biol. Chem., 253, 10.1016/S0021-9258(17)40872-6 Levy, 2000, A neutrophil-derived anti-infective molecule: bactericidal/permeability-increasing protein, Antimicrob. Agents Chemother., 44, 10.1128/AAC.44.11.2925-2931.2000 Carlsson, 2011, Pseudomonas aeruginosa in cystic fibrosis: pyocyanin negative strains are associated with BPI-ANCA and progressive lung disease, J. Cyst. Fibros., 10, 10.1016/j.jcf.2011.03.004 Ulevitch, 1995, Receptor-dependent mechanisms of cell stimulation by bacterial endotoxin, Annu. Rev. Immunol., 13, 10.1146/annurev.iy.13.040195.002253 Rietschel, 1992, Bacterial endotoxins, Sci. Am., 267, 10.1038/scientificamerican0892-54 Wittmann, 2008, Murine bactericidal/permeability-increasing protein inhibits the endotoxic activity of lipopolysaccharide and gram-negative bacteria, J. Immunol., 180, 10.4049/jimmunol.180.11.7546 Elsbach, 1998, The bactericidal/permeability-increasing protein (BPI) in antibacterial host defense, J. Leukoc. Biol., 64, 10.1002/jlb.64.1.14 Mahadeva, 1999, Anti-neutrophil cytoplasmic antibodies (ANCA) against bactericidal/permeability-increasing protein (BPI) and cystic fibrosis lung disease, Clin. Exp. Immunol., 117 Lindberg, 2015, BPI-anca provides additional clinical information to anti- pseudomonas serology: results from a cohort of 117 Swedish cystic fibrosis patients, J. Immunol. Res., 2015, 10.1155/2015/947934 Kobayashi, 2009, Pathogenesis and clinical manifestations of chronic colonization by pseudomonas aeruginosa and its biofilms in the airway tract, J. Infect. Chemother., 15, 10.1007/s10156-008-0691-3 Aebi, 2000, Autoantibodies directed against bactericidal/permeability-increasing protein in patients with cystic fibrosis: association with microbial respiratory tract colonization, Pediatr. Infect. Dis. J., 19, 10.1097/00006454-200003000-00006 Beamer, 1997, Crystal structure of human BPI and two bound phospholipids at 2.4 Angstrom resolution, Science, 80–, 276 Beamer, 2003, Structure of human BPI (bactericidal/permeability-increasing protein) and implications for related proteins, Biochem. Soc. Trans., 31, 10.1042/bst0310791 Bingle, 2000, Characterisation of the human plunc gene, a gene product with an upper airways and nasopharyngeal restricted expression pattern, Biochim. Biophys. Acta Gene Struct. Expr., 1493, 10.1016/S0167-4781(00)00196-2 Calafat, 1998, The bactericidal/permeability-increasing protein (BPI) is present in specific granules of human eosinophils, Blood, 91, 10.1182/blood.V91.12.4770 Reichel, 2003, Bactericidal/permeability-increasing protein is expressed by human dermal fibroblasts and upregulated by interleukin 4, Clin. Diagn. Lab. Immunol., 10 Balakrishnan, 2016, Of men not mice: bactericidal/permeability-increasing protein expressed in human macrophages acts as a phagocytic receptor and modulates entry and replication of gram-negative bacteria, Front. Immunol., 7, 10.3389/fimmu.2016.00455 Canny, 2002, Lipid mediator-induced expression of bactericidal/permeability-increasing protein (BPI) in human mucosal epithelia, Proc. Natl. Acad. Sci. U.S.A., 99, 10.1073/pnas.052533799 Gazzano-Santoro, 1992, High-affinity binding of the bactericidal/permeability-increasing protein and a recombinant amino-terminal fragment to the lipid A region of lipopolysaccharide, Infect. Immun., 60, 10.1128/iai.60.11.4754-4761.1992 Eng Ooi, 1991, Endotoxin-neutralizing properties of the 25 kD N-terminal fragment and a newly isolated 30 kD C-Terminal fragment of the 55-60 kD bactericidal/permeability-increasing protein of human neutrophils, J. Exp. Med., 174 Bülow, 2018, Bactericidal/permeability-increasing protein is an enhancer of bacterial lipoprotein recognition, Front. Immunol., 9, 10.3389/fimmu.2018.02768 Wiese, 1997, Mechanisms of action of bactericidal/permeability-increasing protein BPI on reconstituted outer membranes of gram-negative bacteria, Biochemistry, 36 Beutler, 2003, Innate immune sensing and its roots: the story of endotoxin, Nat. Rev. Immunol., 3, 10.1038/nri1004 Wang, 2010, Endotoxins: lipopolysaccharides of gram-negative bacteria, Subcell. Biochem., 53 Iovine, 1997, An opsonic function of the neutrophil bactericidal/permeability-increasing protein depends on both its N- and C-terminal domains, Proc. Natl. Acad. Sci. U.S.A., 94, 10.1073/pnas.94.20.10973 Iovine, 2002, The carboxyl-terminal domain of closely related endotoxin-binding proteins determines the target of protein-lipopolysaccharide complexes, J. Biol. Chem., 277, 10.1074/jbc.M109622200 Weiss, 1985, Oxygen-independent intracellular and oxygen-dependent extracellular killing of Escherichia coli S15 by human polymorphonuclear leukocytes, J. Clin. Invest., 76, 10.1172/JCI111947 Skopelja, 2016, The role for neutrophil extracellular traps in cystic fibrosis autoimmunity, JCI Insight, 1, 10.1172/jci.insight.88912 Skopelja-Gardner, 2019, Regulation of Pseudomonas aeruginosa-mediated neutrophil extracellular traps, Front. Immunol., 10, 10.3389/fimmu.2019.01670 Elsbach, 1979, Separation and purification of a potent bactericidal/permeability-increasing protein and a closely associated phospholipase A 2 from rabbit polymorphonuclear leukocytes: observations on their relationship, J. Biol. Chem., 254, 10.1016/S0021-9258(19)86622-X Qing, 1996, Lipopolysaccharide binding proteins on polymorphonuclear leukocytes: comparison of adult and neonatal cells, Infect. Immun., 64, 10.1128/iai.64.11.4638-4642.1996 Levy, 1999, Impaired innate immunity in the newborn: newborn neutrophils are deficient in bactericidal/permeability-increasing protein, Pediatrics, 104, 10.1542/peds.104.6.1327 Wright, 1975, Decreased bactericidal activity of leukocytes of stressed newborn infants, Pediatrics, 56, 10.1542/peds.56.4.579 Roos, 2016, Chronic granulomatous disease, Br. Med. Bull., 118, 10.1093/bmb/ldw009 Elsbach, 1998, J. Role of the bactericidal/permeability-increasing protein in host defence, Curr. Opin. Immunol., 10, 10.1016/S0952-7915(98)80030-7 Weiss, 1992, Human bactericidal/permeability-increasing protein and a recombinant NH2-terminal fragment cause killing of serum-resistant gram-negative bacteria in whole blood and inhibit tumor necrosis factor release induced by the bacteria, J. Clin. Invest., 90, 10.1172/JCI115930 Madsen, 1996, Determinants of activation by complement of group II phospholipase A2 acting against Escherichia coli, Infect. Immun., 64, 10.1128/iai.64.7.2425-2430.1996 Mannion, 1990, Separation of sublethal and lethal effects of the bactericidal/permeability increasing protein on Escherichia coli, J. Clin. Invest., 85, 10.1172/JCI114512 Capodici, 1994, Effect of lipopolysaccharide (LPS) chain length on interactions of bactericidal/Permeability-increasing protein and its bioactive 23-kilodalton NH2-terminal fragment with isolated LPS and intact Proteus mirabilis and Escherichia coli, Infect. Immun., 62, 10.1128/iai.62.1.259-265.1994 Gunn, 2001, Bacterial modification of LPS and resistance to antimicrobial peptides, J. Endotoxin Res., 7, 10.1177/09680519010070011001 Horwitz, 1999, Bactericidal/permeability-increasing protein inhibits growth of a strain of Acholeplasma laidlawii and L forms of the gram-positive bacteria Staphylococcus aureus and Streptococcus pyogenes, Antimicrob. Agents Chemother., 43, 10.1128/AAC.43.9.2314 Nishimura, 2001, Bactericidal/permeability-increasing protein promotes complement activation for neutrophil-mediated phagocytosis on bacterial surface, Immunology, 103, 10.1046/j.1365-2567.2001.01263.x Demirdjian, 2020, Distinct contributions of CD18 integrins for binding and phagocytic internalization of Pseudomonas aeruginosa, Infect. Immun., 88, 10.1128/IAI.00011-20 Schultz, 2007, The bactericidal/permeability-increasing protein (BPI) in infection and inflammatory disease, Clin. Chim. Acta, 384, 10.1016/j.cca.2007.07.005 Schumann, 1990, Structure and function of lipopolysaccharide binding protein, Science, 249 Kirschning, 1997, Similar organization of the lipopolysaccharide-binding protein (LBP) and phospholipid transfer protein (PLTP) genes suggests a common gene family of lipid-binding proteins, Genomics, 46, 10.1006/geno.1997.5030 Tobias, 1997, J. Lipopolysaccharide (LPS)-binding proteins BPI and LBP form different types of complexes with LPS, J. Biol. Chem., 272, 10.1074/jbc.272.30.18682 Elsbach, 1993, The bactericidal/permeability-increasing protein (BPI), a potent element in host-defense against gram-negative bacteria and lipopolysaccharide, Immunobiology, 187, 10.1016/S0171-2985(11)80354-2 Dentener, 1993, Antagonistic effects of lipopolysaccharide binding protein and bactericidal/permeability-increasing protein on lipopolysaccharide-induced cytokine release by mononuclear phagocytes: competition for binding to lipopolysaccharide, J. Immunol., 151, 10.4049/jimmunol.151.8.4258 Von der Möhlen, 1995, Inhibition of endotoxin-induced cytokine release and neutrophil activation in humans by use of recombinant bactericidal/permeability-increasing protein, J. Infect. Dis., 172, 10.1093/infdis/172.1.144 Skopelja-Gardner, 2018, NETtling” the host: breaking of tolerance in chronic inflammation and chronic infection, J. Autoimmun., 88, 10.1016/j.jaut.2017.10.008 Hauschild, 1993, ANCA in systemic vasculitides, collagen vascular diseases, rheumatic disorders and inflammatory bowel diseases, Adv. Exp. Med. Biol., 336, 10.1007/978-1-4757-9182-2_36 Van Der Woude, 1985, Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker OF disease activity IN WEGENER’S granulomatosis, Lancet, 325, 10.1016/S0140-6736(85)91147-X Jennette, 1990, Specificity of anti-neutrophil cytoplasmic autoantibodies for proteinase 3 (I), Blood, 75, 10.1182/blood.V75.11.2263.2263 Falk, 1988, Anti-neutrophil cytoplasmic autoantibodies with specificity for myeloperoxidase in patients with systemic vasculitis and idiopathic necrotizing and crescentic glomerulonephritis, N. Engl. J. Med., 318, 10.1056/NEJM198806233182504 Mahadeva, 1997, Vasculitis and bronchiectasis in a patient with antibodies to bactericidal/permeability increasing protein and α1-antitrypsin deficiency, Chest, 112, 10.1378/chest.112.6.1699 Zhao, 1995, Bactericidal/permeability-increasing protein (BPI) is an important antigen for anti-neutrophil cytoplasmic autoantibodies (ANCA) in vasculitis, Clin. Exp. Immunol., 99 Reumaux, 2004, Pathogenesis of diseases associated with antineutrophil cytoplasm autoantibodies, Hum. Immunol., 65, 10.1016/j.humimm.2003.09.013 Söderberg, 2016, Neutrophil extracellular traps in ANCA-associated vasculitis, Front. Immunol., 7, 10.3389/fimmu.2016.00256 Kusunoki, 2016, Peptidylarginine deiminase inhibitor suppresses neutrophil extracellular trap formation and MPO-ANCA production, Front. Immunol., 7, 10.3389/fimmu.2016.00227 Sangaletti, 2012, Neutrophil extracellular traps mediate transfer of cytoplasmic neutrophil antigens to myeloid dendritic cells toward ANCA induction and associated autoimmunity, Blood, 120, 10.1182/blood-2012-03-416156 Roozendaal, 1999, Are anti-neutrophil cytoplasmic antibodies (ANCA) clinically useful in inflammatory bowel disease (IBD)?, Clin. Exp. Immunol., 116 Choi, 2001, Diagnostic performance of antineutrophil cytoplasmic antibody tests for idiopathic vasculitides: metaanalysis with a focus on antimyeloperoxidase antibodies, J. Rheumatol., 28 Zhao, 1996, Autoantibodies against bactericidal/permeability-increasing protein in patients with cystic fibrosis, QJM - Mon. J. Assoc. Physicians, 89 Dorlöchter, 2004, Anti-neutrophil cytoplasmatic antibodies and lung disease in cystic fibrosis, J. Cyst. Fibros., 3, 10.1016/j.jcf.2004.04.005 Walmsley, 1997, Antineutrophil cytoplasm autoantibodies against bactericidal/permeability-increasing protein in inflammatory bowel disease, Gut, 40, 10.1136/gut.40.1.105 Schinke, 2004, Autoantibodies against the bactericidal/permeability-increasing protein from inflammatory bowel disease patients can impair the antibiotic activity of bactericidal/permeability-increasing protein, Inflamm. Bowel Dis., 10, 10.1097/00054725-200411000-00011 Fukuhara, 2013, Systemic vasculitis associated with anti-neutrophil cytoplasmic antibodies against bactericidal/permeability increasing protein, Intern. Med., 52, 10.2169/internalmedicine.52.9477 Schultz, 2000, BPI-ANCA is found in reactive arthritis caused by Yersinia and Salmonella infection and recognise exclusively the C-terminal part of the BPI molecule, Scand. J. Rheumatol., 29 Yang, 1996, Frequency of anti-bactericidal/permeability-increasing protein (BPI) and anti-azurocidin in patients with renal disease, Clin. Exp. Immunol., 105 Stoffel, 1996, Anti-neutrophil cytoplasmic antibodies (ANCA) directed against bactericidal/permeability increasing protein (BPI): a new seromarker for inflammatory bowel disease and associated disorders, Clin. Exp. Immunol., 104 Roozendaal, 1998, Antineutrophil cytoplasmic antibodies in primary sclerosing cholangitis: defined specificities may be associated with distinct clinical features, Am. J. Med., 105, 10.1016/S0002-9343(98)00294-0 Theprungsirikul, 2020, Low-avidity autoantibodies against bactericidal/permeability-increasing protein occur in gram-negative and gram-positive bacteremia, Infect. Immun., 88, 10.1128/IAI.00444-20 Monajemi, 1996, Inflammatory bowel disease is associated with increased mucosal levels of bactericidal/permeability-increasing protein, Gastroenterology, 110, 10.1053/gast.1996.v110.pm8608882 Korkmaz, 2010, Neutrophil elastase, proteinase 3, and cathepsin G as therapeutic targets in human diseases, Pharmacol. Rev., 62, 10.1124/pr.110.002733 Fu, 2018, Extended cleavage specificity of human neutrophil elastase, human proteinase 3, and their distant ortholog clawed frog PR3-three elastases with similar primary but different extended specificities and stability, Front. Immunol., 9, 10.3389/fimmu.2018.02387 Schultz, 2000, Pediatr. Allergy Immunol., 11, 10.1034/j.1399-3038.2000.00069.x Weinrauch, 1995, Extracellular accumulation of potently microbicidal bactericidal/permeability-increasing protein and p15s in an evolving sterile rabbit peritoneal inflammatory exudate, J. Clin. Invest., 95, 10.1172/JCI117873 Šedivá, 2003, Antineutrophil cytoplasmic antibodies directed against bactericidal/permeability-increasing protein detected in children with cystic fibrosis inhibit neutrophil-mediated killing of Pseudomonas aeruginosa, Microb. Infect., 5, 10.1016/S1286-4579(02)00049-7 Schultz, 2003, ANCA against the bactericidal/permeability increasing protein (BPI-ANCA) can compromise the antibiotic function of BPI in a Wegener's granulomatosis patient, Clin. Exp. Rheumatol., 21 Schultz, 2004, BPI-ANCA of pediatric cystic fibrosis patients can impair BPI-mediated killing of E. coli DH5α in vitro, Pediatr. Pulmonol., 37, 10.1002/ppul.10416 Levy, 2003, Expression of BPI (bactericidal/permeability-increasing protein) in human mucosal epithelia, Biochem. Soc. Trans., 31, 795, 10.1042/bst0310795 Haapamäki, 1999, Bactericidal/permeability-increasing protein in colonic mucosa in ulcerative colitis, Hepato-Gastroenterology, 46 Hubacek, 2001, Gene variants of the bactericidal/permeability increasing protein and lipopolysaccharide binding protein in sepsis patients: gender-specific genetic predisposition to sepsis, Crit. Care Med., 29, 10.1097/00003246-200103000-00015 Akidotlessn, 2011, Association between bactericidal/permeability increasing protein (BPI) gene polymorphism (Lys216Glu) and inflammatory bowel disease, J. Crohn’s Colitis, 5 Klein, 2005, A polymorphism of the bactericidal/permeability increasing protein (BPI) gene is associated with Chrohn's disease, J. Clin. Gastroenterol., 39, 10.1097/01.mcg.0000155127.20290.07 Can, 2015, Bactericidal permeability increasing protein gene polymorphism is associated with inflammatory bowel diseases in the Turkish population, Saudi J. Gastroenterol., 21, 10.4103/1319-3767.161642 Bingle, 2011, Distant cousins: genomic and sequence diversity within the BPI fold-containing (BPIF)/PLUNC protein family, Biochem. Soc. Trans., 39, 10.1042/BST0390961 Britto, 2015, Bactericidal/permeability-increasing protein fold-containing family member A1 in airway host protection and respiratory disease, Am. J. Respir. Cell Mol. Biol., 52, 10.1165/rcmb.2014-0297RT Saferali, 2015, Polymorphisms associated with expression of bpifa1/bpifb1 and lung disease severity in cystic fibrosis, Am. J. Respir. Cell Mol. Biol., 53, 10.1165/rcmb.2014-0182OC Saferali, 2020, Immunomodulatory function of the cystic fibrosis modifier gene BPIFA1, PloS One, 15, 10.1371/journal.pone.0227067 Chen, 2012, The role of bactericidal/permeability-increasing protein in men with chronic obstructive pulmonary disease, COPD, 9, 10.3109/15412555.2011.654143 Esnault, 1993, Alphal-antitrypsin genetic polymorphism in ANCA-positive systemic vasculitis, Kidney Int., 43, 10.1038/ki.1993.186 Jagiello, 2005, Association study of Wegener granulomatosis and the functionally relevant A645G polymorphism in the bactericidal/permeability increasing protein (BPI) gene, Int. J. Immunogenet., 32, 10.1111/j.1744-313X.2005.00495.x Horwitz, 1996, Expression and characterization of cysteine-modified variants of an amino-terminal fragment of bactericidal/permeability-increasing protein, Protein Expr. Purif., 8, 10.1006/prep.1996.0071 Vandermeer, 1994, Bactericidal/permeability-increasing protein ameliorates acute lung injury in porcine endotoxemia, J. Appl. Physiol., 76, 10.1152/jappl.1994.76.5.2006 Demetriades, 1999, Bactericidal/permeability-increasing protein (rBPI21) in patients with hemorrhage due to trauma: results of a multicenter phase II clinical trial, J. Trauma Inj. Infect. Crit. Care, 46, 10.1097/00005373-199904000-00018 Levin, 2000, Recombinant bactericidal/permeability-increasing protein (rBPI21) as adjunctive treatment for children with severe meningococcal sepsis: a randomised trial, Lancet, 356, 10.1016/S0140-6736(00)02712-4 Giroir, 1997, Preliminary evaluation of recombinant amino-terminal fragment of human bactericidal/permeability-increasing protein in children with severe meningococcal sepsis, Lancet, 350, 10.1016/S0140-6736(97)06468-4 Levy, 2007, Bactericidal/permeability-increasing protein in host defense and its efficacy in the treatment of bacterial sepsis, Curr. Infect. Dis. Rep., 3, 10.1007/s11908-007-1007-y De Winter, 1995, Recombinant endotoxin-binding protein (rBPI23) attenuates endotoxin- induced circulatory changes in humans, J. Inflamm., 45 Von der Mohlen, 1995, Inhibition of endotoxin-induced activation of the coagulation and fibrinolytic pathways using a recombinant endotoxin-binding protein (rBPI23), Blood, 85, 10.1182/blood.V85.12.3437.bloodjournal85123437 Evans, 1995, Protective effects of a recombinant amino-terminal fragment of human bactericidal/permeability-increasing protein in an animal model of gram-negative sepsis, J. Infect. Dis., 171, 10.1093/infdis/171.1.153 Kelly, 1993, Role of bactericidal permeability-increasing protein in the treatment of gram-negative pneumonia, Surgery, 114 Srivastava, 2007, Recombinant bactericidal/permeability-increasing protein rBPI21 protects against pneumococcal disease, Infect. Immun., 75, 10.1128/IAI.01089-06 Earley, 2015, Burn injury alters the intestinal microbiome and increases gut permeability and bacterial translocation, PloS One, 10, 10.1371/journal.pone.0129996 Hansbrough, 1996, Effects of recombinant bactericidal/permeability-increasing protein (rBPI23) on neutrophil activity in burned rats, J. Trauma Inj. Infect. Crit. Care, 40, 10.1097/00005373-199606000-00005 Pinkenburg, 2016, The human antimicrobial protein bactericidal/permeability-increasing protein (bpi) inhibits the infectivity of influenza a virus, PloS One, 11, 10.1371/journal.pone.0156929 Giroir, 2001, Bactericidal/permeability-increasing protein - lessons learned from the phase III, randomized, clinical trial of rBPI21 for adjunctive treatment of children with severe meningococcemia, vol. 29 Fisher, 1994, Human neutrophil bactericidal/permeability-increasing protein reduces mortality rate from endotoxin challenge: a placebo-controlled study, Crit. Care Med., 22, 10.1097/00003246-199404000-00008 Opal, 1995, Activity of lipopolysaccharide-binding protein-bactericidal/permeability- increasing protein fusion peptide in an experimental model of Pseudomonas sepsis, Antimicrob. Agents Chemother., 39, 10.1128/AAC.39.12.2813 Kong, 2006, BPI700-Fcγy1700 chimeric gene expression and its protective effect in a mice model of the lethal E. coli infection, Chin. Med. J., 119, 10.1097/00029330-200603020-00007 Iwuji, 2019, Prevalence of bactericidal/permeability-increasing protein autoantibodies in cystic fibrosis patients: systematic review and meta-analysis, Pediatric, Allergy, Immunology, and Pulmonology, 32, 10.1089/ped.2018.0970 Tian, 2019, Clinical significance of BPI-ANCA detecting in COPD patients with Pseudomonas aeruginosa colonization, J. Clin. Lab. Anal., 33, 10.1002/jcla.22908 Takeda, 2019, The pathogenicity of BPI-ANCA in a patient with systemic vasculitis, Front. Immunol., 10, 10.3389/fimmu.2019.01334 Langford, 1999, Rare diseases bullet 3: wegener's granulomatosis, Thorax, 54, 10.1136/thx.54.7.629 Steiner, 2009, Autoantibodies against bactericidal/permeability-increasing protein (BPI) in children with acute pneumonia, FEMS Immunol. Med. Microbiol., 57, 10.1111/j.1574-695X.2009.00593.x Theprungsirikul, 2021, Bactericidal/permeability-increasing protein (BPI) preeminently mediates clearance of Pseudomonas aeruginosa in vivo via CD18-dependent phagocytosis, Front. Immunol., 12, 659523, 10.3389/fimmu.2021.659523