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The endotoxin‐binding bactericidal/permeability‐increasing protein (BPI): a target antigen of autoantibodies
Author(s) -
Schultz H.,
Weiss J.,
Carroll S. F.,
Gross W. L.
Publication year - 2001
Publication title -
journal of leukocyte biology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.819
H-Index - 191
eISSN - 1938-3673
pISSN - 0741-5400
DOI - 10.1189/jlb.69.4.505
Subject(s) - autoantibody , proteinase 3 , immunology , biology , inflammation , microbiology and biotechnology , immune system , antibody
The bactericidal/permeability‐increasing protein (BPI) is anendotoxin‐binding neutrophil leukocyte‐granule protein withantibacterial and anti‐endotoxin properties. A recombinant form of BPI(rBPI 21 ) has been developed and is being tested as atherapeutic agent to treat Gram‐negative bacterial infections andexposure to Gram‐negative bacterial endotoxin. BPI is also a targetantigen of anti‐neutrophil cytoplasmic autoantibodies (ANCA). BPI‐ANCAare present in cystic fibrosis, inflammatory bowel disease, vasculitis,and primary sclerosing cholangitis; presence of BPI‐ANCA appearsassociated with a higher inflammatory disease activity and greaterorgan damage. BPI‐ANCA as well as ANCA directed at otherneutrophil‐granule proteins may exacerbate inflammation by nonspecificeffects of extracellular and cell‐associated immune complexes. BPI‐ANCAmay further worsen inflammation by reducing the ability of BPI topromote clearance of Gram‐negative bacteria and bacterial‐associatedendotoxin.