The endotoxin-binding bactericidal/permeability-increasing protein (BPI): A target antigen of autoantibodies

H. Schultz*, J. Weiss, S. F. Carroll, W. L. Gross

*Corresponding author for this work
41 Citations (Scopus)


The 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.

Original languageEnglish
JournalJournal of Leukocyte Biology
Issue number4
Pages (from-to)505-512
Number of pages8
Publication statusPublished - 2001

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)


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