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lüll The endotoxin-binding bactericidal/permeability-increasing protein (BPI): a target antigen of autoantibodies Schultz H; Weiss J; Carroll SF; Gross WLJ Leukoc Biol 2001[Apr]; 69 (4): 505-12The bactericidal/permeability-increasing protein (BPI) is an endotoxin-binding neutrophil leukocyte-granule protein with antibacterial and anti-endotoxin properties. A recombinant form of BPI (rBPI21) has been developed and is being tested as a therapeutic agent to treat gram-negative bacterial infections and exposure to gram-negative bacterial endotoxin. BPI is also a target antigen of anti-neutrophil cytoplasmic autoantibodies (ANCA). BPI-ANCA are present in cystic fibrosis, inflammatory bowel disease, vasculitis, and primary sclerosing cholangitis; presence of BPI-ANCA appears associated with a higher inflammatory disease activity and greater organ damage. BPI-ANCA as well as ANCA directed at other neutrophil-granule proteins may exacerbate inflammation by nonspecific effects of extracellular and cell-associated immune complexes. BPI-ANCA may further worsen inflammation by reducing the ability of BPI to promote clearance of gram-negative bacteria and bacterial-associated endotoxin.|*Membrane Proteins[MESH]|Adult[MESH]|Animals[MESH]|Antibodies, Antineutrophil Cytoplasmic/*immunology[MESH]|Antibody Specificity[MESH]|Antimicrobial Cationic Peptides[MESH]|Autoantigens/*immunology[MESH]|Autoimmune Diseases/immunology[MESH]|Blood Proteins/chemistry/*immunology[MESH]|Child[MESH]|Child, Preschool[MESH]|Clinical Trials, Phase I as Topic[MESH]|Clinical Trials, Phase II as Topic[MESH]|Cytokines/metabolism[MESH]|Cytoplasmic Granules/chemistry[MESH]|Drug Evaluation, Preclinical[MESH]|Endotoxins/metabolism[MESH]|Eosinophils/metabolism[MESH]|Forecasting[MESH]|Gram-Negative Bacterial Infections/drug therapy[MESH]|Humans[MESH]|Infant[MESH]|Inflammation/immunology[MESH]|Mice[MESH]|Multicenter Studies as Topic[MESH]|Neutrophils/metabolism[MESH]|Randomized Controlled Trials as Topic[MESH]|Recombinant Fusion Proteins/immunology/therapeutic use[MESH]|Vasculitis/immunology[MESH] |